Education/CredentialsMedical Degree: Washington UniversityResidency: Children's Hospital - St. LouisFellowship: Children's Hospital and Washington University - St. LouisPostdoctoral Fellowship: Merck, Sharp and Dohme Research Laboratories Contact Information Email strausad@ucmail.uc.edu Research InterestsPediatric Cardiology, Genetic Basis of Pediatric Heart Disease Peer Reviewed Publications Acehan, Devrim; Vaz, Frederic; Houtkooper, Riekelt H; James, Jeanne; Moore, Vicky; Tokunaga, Chonan; Kulik, Willem; Wansapura, Janaka; Toth, Matthew J; Strauss, Arnold; Khuchua, Zaza 2011. Cardiac and skeletal muscle defects in a mouse model of human Barth syndrome. The Journal of biological chemistry, 286 2, 899-908Britigan, Bradley; Strauss, Arnold; Susman, Jeff 2010. Salami science or editorial imperialism? The Journal of pediatrics, 157 3, 518-9; author reply Li, Changhong; Chen, Pan; Palladino, Andrew; Narayan, Srinivas; Russell, Laurie K; Sayed, Samir; Xiong, Guoxiang; Chen, Jie; Stokes, David; Butt, Yasmeen M; Jones, Patricia M; Collins, Heather W; Cohen, Noam A; Cohen, Akiva S; Nissim, Itzhak; Smith, Thomas J; Strauss, Arnold W; Matschinsky, Franz M; Bennett, Michael J; Stanley, Charles A 2010. Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase. The Journal of biological chemistry, 285 41, 31806-18Arnold, Georgianne L; Van Hove, Johan; Freedenberg, Debra; Strauss, Arnold; Longo, Nicola; Burton, Barbara; Garganta, Cheryl; Ficicioglu, Can; Cederbaum, Stephen; Harding, Cary; Boles, Richard G; Matern, Dietrich; Chakraborty, Pranesh; Feigenbaum, Annette 2009. A Delphi clinical practice protocol for the management of very long chain acyl-CoA dehydrogenase deficiency. Molecular genetics and metabolism, 96 3, 85-90Chappell, Tresa; Creech, C Buddy; Parra, David; Strauss, Arnold; Scholl, Frank; Whitney, Gina 2008. Presentation of pulmonary artery intimal sarcoma in an infant with a history of neonatal valvular pulmonic stenosis. The Annals of thoracic surgery, 85 3, 1092-4Hsu, Ho-Wen; Zytkovicz, Thomas H; Comeau, Anne Marie; Strauss, Arnold W; Marsden, Deborah; Shih, Vivian E; Grady, George F; Eaton, Roger B 2008. Spectrum of medium-chain acyl-CoA dehydrogenase deficiency detected by newborn screening. Pediatrics, 121 5, e1108-14Werdich, Andreas A; Baudenbacher, Franz; Dzhura, Igor; Jeyakumar, Loice H; Kannankeril, Prince J; Fleischer, Sidney; LeGrone, Alison; Milatovic, Dejan; Aschner, Michael; Strauss, Arnold W; Anderson, Mark E; Exil, Vernat J 2007. Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice. American journal of physiology. Heart and circulatory physiology, 292 5, H2202-11Werdich,A.A.; Baudenbacher,F.; Dzhura,I.; Jeyakumar,L.H.; Kannankeril,P.J.; Fleischer,S.; Legrone,A.; Milatovic,D.; Aschner,M.; Strauss,A.W.; Anderson,M.; Exil,V.J. 2007. Polymorphic Ventricular Tachycardia and Abnormal Calcium Handling in Very-Long-Chain Acyl-CoA Dehydrogenase Null Mice American Journal of Physiology. Heart and Circulatory Physiology, , Bennett,M.J.; Russell,L.K.; Tokunaga,C.; Narayan,S.B.; Tan,L.; Seegmiller,A.; Boriack,R.L.; Strauss,A.W. 2006. Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase Molecular genetics and metabolism, 89 1-2, 74Djouadi,F.; Aubey,F.; Schlemmer,D.; Gobin,S.; Laforet,P.; Wanders,R.J.; Strauss,A.W.; Bonnefont,J.P.; Bastin,J. 2006. Potential of fibrates in the treatment of fatty acid oxidation disorders: revival of classical drugs? Journal of inherited metabolic disease, 29 2-3, 341Exil, Vernat J; Gardner, Carla D; Rottman, Jeffrey N; Sims, Harold; Bartelds, Beatrijs; Khuchua, Zaza; Sindhal, Rekha; Ni, Gemin; Strauss, Arnold W 2006. Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase. American journal of physiology. Heart and circulatory physiology, 290 3, H1289-97Exil,V.J.; Gardner,C.D.; Rottman,J.N.; Sims,H.; Bartelds,B.; Khuchua,Z.; Sindhal,R.; Ni,G.; Strauss,A.W. 2006. Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase American journal of physiology.Heart and circulatory physiology, 290 3, H1289Hoffman, Jodi D; Steiner, Robert D; Paradise, Lori; Harding, Carey O; Ding, Li; Strauss, Arnold W; Kaplan, Paige 2006. Rhabdomyolysis in the military: recognizing late-onset very long-chain acyl Co-A dehydrogenase deficiency. Military medicine, 171 7, 657-8Hoffman,J.D.; Steiner,R.D.; Paradise,L.; Harding,C.O.; Ding,L.; Strauss,A.W.; Kaplan,P. 2006. Rhabdomyolysis in the military: recognizing late-onset very long-chain acyl Co-A dehydrogenase deficiency Military medicine, 171 7, 657Khuchua, Zaza; Yue, Zou; Batts, Lorene; Strauss, Arnold W 2006. A zebrafish model of human Barth syndrome reveals the essential role of tafazzin in cardiac development and function. Circulation research, 99 2, 201-8Khuchua,Z.; Yue,Z.; Batts,L.; Strauss,A.W. 2006. A zebrafish model of human Barth syndrome reveals the essential role of tafazzin in cardiac development and function Circulation research, 99 2, 201Liebig, Michaela; Schymik, Ina; Mueller, Martina; Wendel, Udo; Mayatepek, Ertan; Ruiter, Jos; Strauss, Arnold W; Wanders, Ronald J A; Spiekerkoetter, Ute 2006. Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels. Pediatrics, 118 3, 1065-9Liebig,M.; Gyenes,M.; Brauers,G.; Ruiter,J.P.; Wendel,U.; Mayatepek,E.; Strauss,A.W.; Wanders,R.J.; Spiekerkoetter,U. 2006. Carnitine supplementation induces long-chain acylcarnitine production--studies in the VLCAD-deficient mouse Journal of inherited metabolic disease, 29 2-3, 343Liebig,M.; Schymik,I.; Mueller,M.; Wendel,U.; Mayatepek,E.; Ruiter,J.; Strauss,A.W.; Wanders,R.J.; Spiekerkoetter,U. 2006. Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels Pediatrics, 118 3, 1065Liske, Michael R; Greeley, Christopher S; Law, David J; Reich, Jonathan D; Morrow, William R; Baldwin, H Scott; Graham, Thomas P; Strauss, Arnold W; Kavanaugh-McHugh, Ann L; Walsh, William F 2006. Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease. Pediatrics, 118 4, e1250-6Liske,M.R.; Greeley,C.S.; Law,D.J.; Reich,J.D.; Morrow,W.R.; Baldwin,H.S.; Graham,T.P.; Strauss,A.W.; Kavanaugh-McHugh,A.L.; Walsh,W.F.; Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease. 2006. Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease Pediatrics, 118 4, e1250Schymik, Ina; Liebig, Michaela; Mueller, Martina; Wendel, Udo; Mayatepek, Ertan; Strauss, Arnold W; Wanders, Ronald J A; Spiekerkoetter, Ute 2006. Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry. The Journal of pediatrics, 149 1, 128-30Schymik,I.; Liebig,M.; Mueller,M.; Wendel,U.; Mayatepek,E.; Strauss,A.W.; Wanders,R.J.; Spiekerkoetter,U. 2006. Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry The Journal of pediatrics, 149 1, 128Spiekerkoetter,U.; Ruiter,J.; Tokunaga,C.; Wendel,U.; Mayatepek,E.; Wijburg,F.A.; Strauss,A.W.; Wanders,R.J. 2006. Evidence for impaired gluconeogenesis in very long-chain acyl-CoA dehydrogenase-deficient mice Hormone and metabolic research.Hormon- und Stoffwechselforschung.Hormones et metabolisme, 38 10, 625Djouadi,F.; Aubey,F.; Schlemmer,D.; Ruiter,J.P.; Wanders,R.J.; Strauss,A.W.; Bastin,J. 2005. Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders Human molecular genetics, 14 18, 2695Shekhawat, Prem S; Matern, Dietrich; Strauss, Arnold W 2005. Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and management. Pediatric research, 57 5 Pt 2, 78R-86RShekhawat,P.S.; Matern,D.; Strauss,A.W. 2005. Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and management Pediatric research, 57 5 Pt 2, 78RSpiekerkoetter, Ute; Tokunaga, Chonan; Wendel, Udo; Mayatepek, Ertan; Ijlst, Lodewijk; Vaz, Frederic M; van Vlies, Naomi; Overmars, Henk; Duran, Marinus; Wijburg, Frits A; Wanders, Ronald J; Strauss, Arnold W 2005. Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. Pediatric research, 57 6, 760-4Spiekerkoetter,U.; Tokunaga,C.; Wendel,U.; Mayatepek,E.; Ijlst,L.; Vaz,F.M.; van Vlies,N.; Overmars,H.; Duran,M.; Wijburg,F.A.; Wanders,R.J.; Strauss,A.W. 2005. Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice Pediatric research, 57 6, 760Strauss, Arnold W 2005. Surprising? Perhaps not. Long-chain fatty acid oxidation during human fetal development. Pediatric research, 57 6, 753-4Strauss,A.W. 2005. Surprising? Perhaps not. Long-chain fatty acid oxidation during human fetal development Pediatric research, 57 6, 753McKinney,J.T.; Longo,N.; Hahn,S.H.; Matern,D.; Rinaldo,P.; Strauss,A.W.; Dobrowolski,S.F. 2004. Rapid, comprehensive screening of the human medium chain acyl-CoA dehydrogenase gene Molecular genetics and metabolism, 82 2, 112Spiekerkoetter, Ute; Bennett, Michael J; Ben-Zeev, Bruria; Strauss, Arnold W; Tein, Ingrid 2004. Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein. Muscle & nerve, 29 1, 66-72Spiekerkoetter, Ute; Khuchua, Zaza; Yue, Zou; Bennett, Michael J; Strauss, Arnold W 2004. General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnover. Pediatric research, 55 2, 190-6Spiekerkoetter,U.; Bennett,M.J.; Ben-Zeev,B.; Strauss,A.W.; Tein,I. 2004. Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein Muscle & nerve, 29 1, 66Spiekerkoetter,U.; Khuchua,Z.; Yue,Z.; Bennett,M.J.; Strauss,A.W. 2004. General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnover Pediatric research, 55 2, 190Spiekerkoetter,U.; Tokunaga,C.; Wendel,U.; Mayatepek,E.; Exil,V.; Duran,M.; Wijburg,F.A.; Wanders,R.J.; Strauss,A.W. 2004. Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress European journal of clinical investigation, 34 3, 191Spierkerkoetter,U.; Khuchua,Z.; Yue,Z.; Strauss,A.W. 2004. The early-onset phenotype of mitochondrial trifunctional protein deficiency: a lethal disorder with multiple tissue involvement Journal of inherited metabolic disease, 27 2, 294Strauss, Arnold W 2004. Tandem mass spectrometry in discovery of disorders of the metabolome. The Journal of clinical investigation, 113 3, 354-6Strauss,A.W. 2004. Tandem mass spectrometry in discovery of disorders of the metabolome The Journal of clinical investigation, 113 3, 354Tsirka, Anna E; Trinkaus, Kathryn; Chen, Su-Chiung; Lipshultz, Steven E; Towbin, Jeffrey A; Colan, Steven D; Exil, Vernat; Strauss, Arnold W; Canter, Charles E 2004. Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation. Journal of the American College of Cardiology, 44 2, 391-7Tsirka,A.E.; Trinkaus,K.; Chen,S.C.; Lipshultz,S.E.; Towbin,J.A.; Colan,S.D.; Exil,V.; Strauss,A.W.; Canter,C.E. 2004. Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation Journal of the American College of Cardiology, 44 2, 391Tyni, Tiina; Paetau, Anders; Strauss, Arnold W; Middleton, Bruce; Kivelä, Tero 2004. Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Pediatric research, 56 5, 744-50Tyni,T.; Paetau,A.; Strauss,A.W.; Middleton,B.; Kivela,T. 2004. Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency Pediatric research, 56 5, 744Boero,J.; Qin,W.; Cheng,J.; Woolsey,T.A.; Strauss,A.W.; Khuchua,Z. 2003. Restricted neuronal expression of ubiquitous mitochondrial creatine kinase: changing patterns in development and with increased activity Molecular and cellular biochemistry, 244 1-2, 69Exil, Vernat J; Roberts, Richard L; Sims, Harold; McLaughlin, Jacquelin E; Malkin, Robert A; Gardner, Carla D; Ni, Gemin; Rottman, Jeffrey N; Strauss, Arnold W 2003. Very-long-chain acyl-coenzyme a dehydrogenase deficiency in mice. Circulation research, 93 5, 448-55Exil,V.J.; Roberts,R.L.; Sims,H.; McLaughlin,J.E.; Malkin,R.A.; Gardner,C.D.; Ni,G.; Rottman,J.N.; Strauss,A.W. 2003. Very-long-chain acyl-coenzyme a dehydrogenase deficiency in mice Circulation research, 93 5, 448Khuchua,Z.; Wozniak,D.F.; Bardgett,M.E.; Yue,Z.; McDonald,M.; Boero,J.; Hartman,R.E.; Sims,H.; Strauss,A.W. 2003. Deletion of the N-terminus of murine map2 by gene targeting disrupts hippocampal ca1 neuron architecture and alters contextual memory Neuroscience, 119 1, 101Lee,J.E.; Yoon,H.R.; Paik,K.H.; Hwang,S.J.; Shim,J.W.; Chang,Y.S.; Park,W.S.; Strauss,A.W.; Jin,D.K. 2003. A case of mitochondrial trifunctional protein deficiency diagnosed by acylcarnitine profile and DNA analysis in a dried blood spot of a 4-day-old boy Journal of inherited metabolic disease, 26 4, 403Shekhawat, Prem; Bennett, Michael J; Sadovsky, Yoel; Nelson, D Michael; Rakheja, Dinesh; Strauss, Arnold W 2003. Human placenta metabolizes fatty acids: implications for fetal fatty acid oxidation disorders and maternal liver diseases. American journal of physiology. Endocrinology and metabolism, 284 6, E1098-105Shekhawat,P.; Bennett,M.J.; Sadovsky,Y.; Nelson,D.M.; Rakheja,D.; Strauss,A.W. 2003. Human placenta metabolizes fatty acids: implications for fetal fatty acid oxidation disorders and maternal liver diseases American journal of physiology.Endocrinology and metabolism, 284 6, E1098Spiekerkoetter, Ute; Sun, Bin; Khuchua, Zaza; Bennett, Michael J; Strauss, Arnold W 2003. Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations. Human mutation, 21 6, 598-607Spiekerkoetter, Ute; Sun, Bin; Zytkovicz, Thomas; Wanders, Ronald; Strauss, Arnold W; Wendel, Udo 2003. MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency. The Journal of pediatrics, 143 3, 335-42Spiekerkoetter,U.; Sun,B.; Khuchua,Z.; Bennett,M.J.; Strauss,A.W. 2003. Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations Human mutation, 21 6, 598Spiekerkoetter,U.; Sun,B.; Zytkovicz,T.; Wanders,R.; Strauss,A.W.; Wendel,U. 2003. MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency The Journal of pediatrics, 143 3, 335Strauss, Arnold; Lock, James E 2003. Pediatric cardiomyopathy--a long way to go. The New England journal of medicine, 348 17, 1703-5Hintz, Susan R; Matern, Dietrich; Strauss, Arnold; Bennett, Michael J; Hoyme, H Eugene; Schelley, Susan; Kobori, Joyce; Colby, Christopher; Lehman, Norman L; Enns, Gregory M 2002. Early neonatal diagnosis of long-chain 3-hydroxyacyl coenzyme a dehydrogenase and mitochondrial trifunctional protein deficiencies. Molecular genetics and metabolism, 75 2, 120-7Spiekerkoetter, Ute; Eeds, Angela; Yue, Zou; Haines, Jonathan; Strauss, Arnold W; Summar, Marshall 2002. Uniparental disomy of chromosome 2 resulting in lethal trifunctional protein deficiency due to homozygous alpha-subunit mutations. Human mutation, 20 6, 447-51Spiekerkoetter,U.; Eeds,A.; Yue,Z.; Haines,J.; Strauss,A.W.; Summar,M. 2002. Uniparental disomy of chromosome 2 resulting in lethal trifunctional protein deficiency due to homozygous alpha-subunit mutations Human mutation, 20 6, 447Straussberg, Rachel; Strauss, Arnold W 2002. A novel mutation of late-onset very-long-chain acyl-CoA dehydrogenase deficiency. Pediatric neurology, 27 2, 136-7Straussberg,R.; Strauss,A.W. 2002. A novel mutation of late-onset very-long-chain acyl-CoA dehydrogenase deficiency Pediatric neurology, 27 2, 136Yang, Zi; Yamada, Jennifer; Zhao, Yiwen; Strauss, Arnold W; Ibdah, Jamal A 2002. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease. JAMA : the journal of the American Medical Association, 288 17, 2163-6Yang, Zi; Zhao, Yiwen; Bennett, Michael J; Strauss, Arnold W; Ibdah, Jamal A 2002. Fetal genotypes and pregnancy outcomes in 35 families with mitochondrial trifunctional protein mutations. American journal of obstetrics and gynecology, 187 3, 715-20Yang,Z.; Yamada,J.; Zhao,Y.; Strauss,A.W.; Ibdah,J.A. 2002. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease JAMA : the journal of the American Medical Association, 288 17, 2163Yang,Z.; Zhao,Y.; Bennett,M.J.; Strauss,A.W.; Ibdah,J.A. 2002. Fetal genotypes and pregnancy outcomes in 35 families with mitochondrial trifunctional protein mutations American Journal of Obstetrics and Gynecology, 187 3, 715Albers,S.; Levy,H.L.; Irons,M.; Strauss,A.W.; Marsden,D. 2001. Compound heterozygosity in four asymptomatic siblings with medium-chain acyl-CoA dehydrogenase deficiency Journal of inherited metabolic disease, 24 3, 417Barycki,J.J.; O'Brien,L.K.; Strauss,A.W.; Banaszak,L.J. 2001. Glutamate 170 of human l-3-hydroxyacyl-CoA dehydrogenase is required for proper orientation of the catalytic histidine and structural integrity of the enzyme The Journal of biological chemistry, 276 39, 36718Ibdah,J.A.; Paul,H.; Zhao,Y.; Binford,S.; Salleng,K.; Cline,M.; Matern,D.; Bennett,M.J.; Rinaldo,P.; Strauss,A.W. 2001. Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death The Journal of clinical investigation, 107 11, 1403Ibdah,J.A.; Zhao,Y.; Viola,J.; Gibson,B.; Bennett,M.J.; Strauss,A.W. 2001. Molecular prenatal diagnosis in families with fetal mitochondrial trifunctional protein mutations The Journal of pediatrics, 138 3, 396Jones,P.M.; Moffitt,M.; Joseph,D.; Harthcock,P.A.; Boriack,R.L.; Ibdah,J.A.; Strauss,A.W.; Bennett,M.J. 2001. Accumulation of free 3-hydroxy fatty acids in the culture media of fibroblasts from patients deficient in long-chain l-3-hydroxyacyl-CoA dehydrogenase: a useful diagnostic aid Clinical chemistry, 47 7, 1190Matern,D.; Schehata,B.M.; Shekhawa,P.; Strauss,A.W.; Bennett,M.J.; Rinaldo,P. 2001. Placental floor infarction complicating the pregnancy of a fetus with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency Molecular genetics and metabolism, 72 3, 265Wood,J.C.; Magera,M.J.; Rinaldo,P.; Seashore,M.R.; Strauss,A.W.; Friedman,A. 2001. Diagnosis of very long chain acyl-dehydrogenase deficiency from an infant's newborn screening card Pediatrics, 108 1, E19Woodside,K.J.; Knisely,A.S.; Strauss,A.W.; Gugliuzza,K.K.; Daller,J.A. 2001. Progression of hepatic damage during cold storage after procurement in a liver and kidney donor with HELLP syndrome Transplantation, 72 12, 1990Yoon,H.R.; Strauss,A.W.; Yoo,H.W. 2001. Sudden death in a Korean infant with very long-chain acyl-CoA dehydrogenase deficiency Journal of inherited metabolic disease, 24 3, 407Zytkovicz,T.H.; Fitzgerald,E.F.; Marsden,D.; Larson,C.A.; Shih,V.E.; Johnson,D.M.; Strauss,A.W.; Comeau,A.M.; Eaton,R.B.; Grady,G.F. 2001. Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program Clinical chemistry, 47 11, 1945Barycki,J.J.; O'Brien,L.K.; Strauss,A.W.; Banaszak,L.J. 2000. Sequestration of the active site by interdomain shifting. Crystallographic and spectroscopic evidence for distinct conformations of L-3-hydroxyacyl-CoA dehydrogenase The Journal of biological chemistry, 275 35, 27186Bennett,M.J.; Rinaldo,P.; Strauss,A.W. 2000. Inborn errors of mitochondrial fatty acid oxidation Critical reviews in clinical laboratory sciences, 37 1, 1Schlattner,U.; Eder,M.; Dolder,M.; Khuchua,Z.A.; Strauss,A.W.; Wallimann,T. 2000. Divergent enzyme kinetics and structural properties of the two human mitochondrial creatine kinase isoenzymes Biological chemistry, 381 11, 1063Barycki,J.J.; O'Brien,L.K.; Birktoft,J.J.; Strauss,A.W.; Banaszak,L.J. 1999. Pig heart short chain L-3-hydroxyacyl-CoA dehydrogenase revisited: sequence analysis and crystal structure determination Protein science : a publication of the Protein Society, 8 10, 2010Barycki,J.J.; O'Brien,L.K.; Bratt,J.M.; Zhang,R.; Sanishvili,R.; Strauss,A.W.; Banaszak,L.J. 1999. Biochemical characterization and crystal structure determination of human heart short chain L-3-hydroxyacyl-CoA dehydrogenase provide insights into catalytic mechanism Biochemistry, 38 18, 5786Hahn,S.H.; Lee,E.H.; Jung,J.W.; Hong,C.H.; Yoon,H.R.; Rinaldo,P.; Sims,H.; Gibson,B.; Strauss,A.W. 1999. Very long chain acyl coenzyme A dehydrogenase deficiency in a 5-month-old Korean boy: identification of a novel mutation The Journal of pediatrics, 135 2 Pt 1, 250Ibdah,J.A.; Bennett,M.J.; Rinaldo,P.; Zhao,Y.; Gibson,B.; Sims,H.F.; Strauss,A.W. 1999. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women The New England journal of medicine, 340 22, 1723Ibdah,J.A.; Dasouki,M.J.; Strauss,A.W. 1999. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: variable expressivity of maternal illness during pregnancy and unusual presentation with infantile cholestasis and hypocalcaemia Journal of inherited metabolic disease, 22 7, 811Matern,D.; Strauss,A.W.; Hillman,S.L.; Mayatepek,E.; Millington,D.S.; Trefz,F.K. 1999. Diagnosis of mitochondrial trifunctional protein deficiency in a blood spot from the newborn screening card by tandem mass spectrometry and DNA analysis Pediatric research, 46 1, 45Mathur,A.; Sims,H.F.; Gopalakrishnan,D.; Gibson,B.; Rinaldo,P.; Vockley,J.; Hug,G.; Strauss,A.W. 1999. Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death Circulation, 99 10, 1337Qin,W.; Khuchua,Z.; Boero,J.; Payne,R.M.; Strauss,A.W. 1999. Oxidative myocytes of heart and skeletal muscle express abundant sarcomeric mitochondrial creatine kinase The Histochemical journal, 31 6, 357Strauss,A.W.; Bennett,M.J.; Rinaldo,P.; Sims,H.F.; O'Brien,L.K.; Zhao,Y.; Gibson,B.; Ibdah,J. 1999. Inherited long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and a fetal-maternal interaction cause maternal liver disease and other pregnancy complications Seminars in perinatology, 23 2, 100Zhou,Y.; Kelly,D.P.; Strauss,A.W.; Sims,H.; Zhang,Z. 1999. Characterization of the human very-long-chain acyl-CoA dehydrogenase gene promoter region: a role for activator protein 2 Molecular genetics and metabolism, 68 4, 481Benson,D.W.; Sharkey,A.; Fatkin,D.; Lang,P.; Basson,C.T.; McDonough,B.; Strauss,A.W.; Seidman,J.G.; Seidman,C.E. 1998. Reduced penetrance, variable expressivity, and genetic heterogeneity of familial atrial septal defects Circulation, 97 20, 2043Ibdah,J.A.; Tein,I.; Dionisi-Vici,C.; Bennett,M.J.; IJlst,L.; Gibson,B.; Wanders,R.J.; Strauss,A.W. 1998. Mild trifunctional protein deficiency is associated with progressive neuropathy and myopathy and suggests a novel genotype-phenotype correlation The Journal of clinical investigation, 102 6, 1193Khuchua,Z.A.; Qin,W.; Boero,J.; Cheng,J.; Payne,R.M.; Saks,V.A.; Strauss,A.W. 1998. Octamer formation and coupling of cardiac sarcomeric mitochondrial creatine kinase are mediated by charged N-terminal residues The Journal of biological chemistry, 273 36, 22990Qin,W.; Khuchua,Z.; Cheng,J.; Boero,J.; Payne,R.M.; Strauss,A.W. 1998. Molecular characterization of the creatine kinases and some historical perspectives Molecular and cellular biochemistry, 184 1-2, 153Strauss, Arnold W. 1998. The molecular basis of congenital cardiac disease. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual, 1 , 179-188Strauss,A.W. 1998. The molecular basis of congenital cardiac disease , 1 , 179Eder,M.; Krautle,F.; Dong,Y.; Vock,P.; Kieweg,V.; Kim,J.J.; Strauss,A.W.; Ghisla,S. 1997. Characterization of human and pig kidney long-chain-acyl-CoA dehydrogenases and their role in beta-oxidation European journal of biochemistry / FEBS, 245 3, 600Qin,W.; Khuchua,Z.; Klein,S.C.; Strauss,A.W. 1997. Elements regulating cardiomyocyte expression of the human sarcomeric mitochondrial creatine kinase gene in transgenic mice The Journal of biological chemistry, 272 40, 25210Zhang,Z.; Zhou,Y.; Mendelsohn,N.J.; Bauer,G.S.; Strauss,A.W. 1997. Regulation of the human long chain acyl-CoA dehydrogenase gene by nuclear hormone receptor transcription factors Biochimica et biophysica acta, 1350 1, 53Bromberg,B.I.; Mazziotti,M.V.; Canter,C.E.; Spray,T.L.; Strauss,A.W.; Foglia,R.P. 1996. Recognition and management of nonpenetrating cardiac trauma in children The Journal of pediatrics, 128 4, 536Isaacs,J.D.,Jr; Sims,H.F.; Powell,C.K.; Bennett,M.J.; Hale,D.E.; Treem,W.R.; Strauss,A.W. 1996. Maternal acute fatty liver of pregnancy associated with fetal trifunctional protein deficiency: molecular characterization of a novel maternal mutant allele Pediatric research, 40 3, 393Johnson,M.C.; Watson,M.S.; Strauss,A.W. 1996. Chromosome 22q11 monosomy and the genetic basis of congenital heart disease The Journal of pediatrics, 129 1, 1Strauss,A.W.; Johnson,M.C. 1996. The genetic basis of pediatric cardiovascular disease Seminars in perinatology, 20 6, 564Brackett,J.C.; Sims,H.F.; Rinaldo,P.; Shapiro,S.; Powell,C.K.; Bennett,M.J.; Strauss,A.W. 1995. Two alpha subunit donor splice site mutations cause human trifunctional protein deficiency The Journal of clinical investigation, 95 5, 2076Johnson,M.C.; Payne,R.M.; Grant,J.W.; Strauss,A.W. 1995. The genetic basis of paediatric heart disease Annals of Medicine, 27 3, 289Johnson,M.C.; Strauss,A.W.; Dowton,S.B.; Spray,T.L.; Huddleston,C.B.; Wood,M.K.; Slaugh,R.A.; Watson,M.S. 1995. Deletion within chromosome 22 is common in patients with absent pulmonary valve syndrome The American Journal of Cardiology, 76 1, 66Johnson,M.C.; Watson,M.S.; Strauss,A.W.; Spray,T.L. 1995. Anomalous origin of the right pulmonary artery from the aorta and CATCH 22 syndrome The Annals of Thoracic Surgery, 60 3, 681Leone,T.C.; Cresci,S.; Carter,M.E.; Zhang,Z.; Lala,D.S.; Strauss,A.W.; Kelly,D.P. 1995. The human medium chain Acyl-CoA dehydrogenase gene promoter consists of a complex arrangement of nuclear receptor response elements and Sp1 binding sites The Journal of biological chemistry, 270 27, 16308Leone,T.C.; Cresci,S.; Carter,M.E.; Zhang,Z.; Lala,D.S.; Strauss,A.W.; Kelly,D.P. 1995. The human medium chain acyl-CoA dehydrogenase gene promoter consists of a complex arrangement of nuclear receptor response elements and Sp1 binding sites The Journal of biological chemistry, 270 41, 24622Payne,R.M.; Johnson,M.C.; Grant,J.W.; Strauss,A.W. 1995. Toward a molecular understanding of congenital heart disease Circulation, 91 2, 494Peterson,K.L.; Sergienko,E.E.; Wu,Y.; Kumar,N.R.; Strauss,A.W.; Oleson,A.E.; Muhonen,W.W.; Shabb,J.B.; Srivastava,D.K. 1995. Recombinant human liver medium-chain acyl-CoA dehydrogenase: purification, characterization, and the mechanism of interactions with functionally diverse C8-CoA molecules Biochemistry, 34 45, 14942Sims,H.F.; Brackett,J.C.; Powell,C.K.; Treem,W.R.; Hale,D.E.; Bennett,M.J.; Gibson,B.; Shapiro,S.; Strauss,A.W. 1995. The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy Proceedings of the National Academy of Sciences of the United States of America, 92 3, 841Strauss,A.W.; Powell,C.K.; Hale,D.E.; Anderson,M.M.; Ahuja,A.; Brackett,J.C.; Sims,H.F. 1995. Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood Proceedings of the National Academy of Sciences of the United States of America, 92 23, 10496Weinberger,M.J.; Rinaldo,P.; Strauss,A.W.; Bennett,M.J. 1995. Intact alpha-subunit is required for membrane-binding of human mitochondrial trifunctional beta-oxidation protein, but is not necessary for conferring 3-ketoacyl-CoA thiolase activity to the beta-subunit Biochemical and biophysical research communications, 209 1, 47Ziadeh,R.; Hoffman,E.P.; Finegold,D.N.; Hoop,R.C.; Brackett,J.C.; Strauss,A.W.; Naylor,E.W. 1995. Medium chain acyl-CoA dehydrogenase deficiency in Pennsylvania: neonatal screening shows high incidence and unexpected mutation frequencies Pediatric research, 37 5, 675Brackett,J.C.; Sims,H.F.; Steiner,R.D.; Nunge,M.; Zimmerman,E.M.; deMartinville,B.; Rinaldo,P.; Slaugh,R.; Strauss,A.W. 1994. A novel mutation in medium chain acyl-CoA dehydrogenase causes sudden neonatal death The Journal of clinical investigation, 94 4, 1477Djordjevic,S.; Dong,Y.; Paschke,R.; Frerman,F.E.; Strauss,A.W.; Kim,J.J. 1994. Identification of the catalytic base in long chain acyl-CoA dehydrogenase Biochemistry, 33 14, 4258Johnson,M.C.; Strauss,A.W. 1994. The jury is still out regarding balloon therapy for native aortic coarctation Journal of the American College of Cardiology, 24 6, 1589Kelly,D.P.; Strauss,A.W. 1994. Inherited cardiomyopathies The New England journal of medicine, 330 13, 913O'Shea,D.L.; Gast,M.J.; Murdock,G.L.; Payne,R.M.; Strauss,A.W. 1994. Expression of engineered human 17 beta-estradiol dehydrogenase in a prokaryotic system Journal of the Society for Gynecologic Investigation, 1 2, 143Payne,R.M.; Strauss,A.W. 1994. Developmental expression of sarcomeric and ubiquitous mitochondrial creatine kinase is tissue-specific Biochimica et biophysica acta, 1219 1, 33Payne,R.M.; Strauss,A.W. 1994. Expression of the mitochondrial creatine kinase genes Molecular and cellular biochemistry, 133-134 , 235Andresen,B.S.; Kolvraa,S.; Bross,P.; Bolund,L.; Curtis,D.; Eiberg,H.; Zhang,Z.; Kelly,D.P.; Strauss,A.W.; Gregersen,N. 1993. A silent A to G mutation in exon 11 of the medium-chain acyl-CoA dehydrogenase (MCAD) gene Human molecular genetics, 2 4, 488Hainline,B.E.; Kahlenbeck,D.J.; Grant,J.; Strauss,A.W. 1993. Tissue specific and developmental expression of rat long-and medium-chain acyl-CoA dehydrogenases Biochimica et biophysica acta, 1216 3, 460Johnson,M.C.; Canter,C.E.; Strauss,A.W.; Spray,T.L. 1993. Repair of coarctation of the aorta in infancy: comparison of surgical and balloon angioplasty American Heart Journal, 125 2 Pt 1, 464Payne,R.M.; Friedman,D.L.; Grant,J.W.; Perryman,M.B.; Strauss,A.W. 1993. Creatine kinase isoenzymes are highly regulated during pregnancy in rat uterus and placenta The American Journal of Physiology, 265 4 Pt 1, E624Whelan,A.J.; Strauss,A.W.; Hale,D.E.; Mendelsohn,N.J.; Kelly,D.P. 1993. Expression and characterization of human mutant (glutamic acid304) medium-chain acyl-coenzyme A dehydrogenase in mammalian cells Pediatric research, 34 5, 694Zhang,Z.; Kolvraa,S.; Zhou,Y.; Kelly,D.P.; Gregersen,N.; Strauss,A.W. 1993. Three RFLPs defining a haplotype associated with the common mutation in human medium-chain acyl-CoA dehydrogenase (MCAD) deficiency occur in Alu repeats American Journal of Human Genetics, 52 6, 1111Ghisla,S.; Engst,S.; Moll,M.; Bross,P.; Strauss,A.W.; Kim,J.J. 1992. Alpha, beta-dehydrogenation by acyl-CoA dehydrogenases: role of functional groups at the active center Progress in clinical and biological research, 375 , 127Kelly,D.P.; Hale,D.E.; Rutledge,S.L.; Ogden,M.L.; Whelan,A.J.; Zhang,Z.; Strauss,A.W. 1992. Molecular basis of inherited medium-chain acyl-CoA dehydrogenase deficiency causing sudden child death Journal of inherited metabolic disease, 15 2, 171Kelly,D.P.; Whelan,A.J.; Hale,D.E.; Rinaldo,P.; Rutledge,S.L.; Zhang,Z.; Strauss,A.W. 1992. Molecular characterization of medium-chain acyl-CoA dehydrogenase deficiency causing sudden death Progress in clinical and biological research, 375 , 463Kelly,D.P.; Zhang,Z.; Raisher,B.D.; Ogden,M.L.; Strauss,A.W. 1992. Identification and characterization of the 5' regulatory region of the human medium-chain acyl-CoA dehydrogenase (MCAD) gene Progress in clinical and biological research, 375 , 143Raisher,B.D.; Grant,J.W.; Martin,T.C.; Strauss,A.W.; Spray,T.L. 1992. Complete repair of total anomalous pulmonary venous connection in infancy The Journal of thoracic and cardiovascular surgery, 104 2, 443Raisher,B.D.; Gulick,T.; Zhang,Z.; Strauss,A.W.; Moore,D.D.; Kelly,D.P. 1992. Identification of a novel retinoid-responsive element in the promoter region of the medium chain acyl-coenzyme A dehydrogenase gene The Journal of biological chemistry, 267 28, 20264Su,C.Y.; Payne,M.; Strauss,A.W.; Dillmann,W.H. 1992. Selective reduction of creatine kinase subunit mRNAs in striated muscle of diabetic rats The American Journal of Physiology, 263 2 Pt 1, E310Tanaka,K.; Yokota,I.; Coates,P.M.; Strauss,A.W.; Kelly,D.P.; Zhang,Z.; Gregersen,N.; Andresen,B.S.; Matsubara,Y.; Curtis,D. 1992. Mutations in the medium chain acyl-CoA dehydrogenase (MCAD) gene Human mutation, 1 4, 271Zhang,Z.F.; Kelly,D.P.; Kim,J.J.; Zhou,Y.Q.; Ogden,M.L.; Whelan,A.J.; Strauss,A.W. 1992. Structural organization and regulatory regions of the human medium-chain acyl-CoA dehydrogenase gene Biochemistry, 31 1, 81Fontanet,H.L.; Trask,R.V.; Haas,R.C.; Strauss,A.W.; Abendschein,D.R.; Billadello,J.J. 1991. Regulation of expression of M, B, and mitochondrial creatine kinase mRNAs in the left ventricle after pressure overload in rats Circulation research, 68 4, 1007Gregersen,N.; Andresen,B.S.; Bross,P.; Winter,V.; Rudiger,N.; Engst,S.; Christensen,E.; Kelly,D.; Strauss,A.W.; Kolvraa,S. 1991. Molecular characterization of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency: identification of a lys329 to glu mutation in the MCAD gene, and expression of inactive mutant enzyme protein in E. coli Human genetics, 86 6, 545Gregersen,N.; Andresen,B.S.; Bross,P.; Winter,V.; Rudiger,N.; Engst,S.; Ghisla,S.; Christensen,E.; Kelly,D.; Strauss,A.W. 1991. Characterization of a disease-causing Lys329 to Glu mutation in 16 patients with medium-chain acyl-CoA dehydrogenase deficiency Journal of inherited metabolic disease, 14 3, 314Klein,S.C.; Haas,R.C.; Perryman,M.B.; Billadello,J.J.; Strauss,A.W. 1991. Regulatory element analysis and structural characterization of the human sarcomeric mitochondrial creatine kinase gene The Journal of biological chemistry, 266 27, 18058Murphy,A.M.; Jones,L.,2nd; Sims,H.F.; Strauss,A.W. 1991. Molecular cloning of rat cardiac troponin I and analysis of troponin I isoform expression in developing rat heart Biochemistry, 30 3, 707Payne,R.M.; Haas,R.C.; Strauss,A.W. 1991. Structural characterization and tissue-specific expression of the mRNAs encoding isoenzymes from two rat mitochondrial creatine kinase genes Biochimica et biophysica acta, 1089 3, 352Shackelford,P.G.; Strauss,A.W. 1991. Kawasaki syndrome The New England journal of medicine, 324 23, 1664Agrawal,H.C.; Agrawal,D.; Strauss,A.W. 1990. Cleavage of the P0 glycoprotein of the rat peripheral nerve myelin: tentative identification of cleavage site and evidence for the precursor-product relationship Neurochemical research, 15 10, 993Bross,P.; Engst,S.; Strauss,A.W.; Kelly,D.P.; Rasched,I.; Ghisla,S. 1990. Characterization of wild-type and an active site mutant of human medium chain acyl-CoA dehydrogenase after expression in Escherichia coli The Journal of biological chemistry, 265 13, 7116Haas,R.C.; Strauss,A.W. 1990. Separate nuclear genes encode sarcomere-specific and ubiquitous human mitochondrial creatine kinase isoenzymes The Journal of biological chemistry, 265 12, 6921Kelly,D.P.; Strauss,A.W. 1990. The tissue-specific and developmental regulation of expression of rat medium-chain acyl-CoA dehydrogenase mRNA Progress in clinical and biological research, 321 , 599Kelly,D.P.; Whelan,A.J.; Ogden,M.L.; Alpers,R.; Zhang,Z.F.; Bellus,G.; Gregersen,N.; Dorland,L.; Strauss,A.W. 1990. Molecular characterization of inherited medium-chain acyl-CoA dehydrogenase deficiency Proceedings of the National Academy of Sciences of the United States of America, 87 23, 9236Long,C.A.; Bauer,G.S.; Lowe,M.E.; Strauss,A.W.; Gast,M.J. 1990. Isolation and characterization of the gene from a human genome encoding 17 beta-estradiol dehydrogenase: a comparison of Jar and BeWo choriocarcinoma cell lines American Journal of Obstetrics and Gynecology, 163 6 Pt 1, 1976Lowe,M.; Strauss,A.W.; Alpers,R.; Seetharam,S.; Alpers,D.H. 1990. Molecular cloning and expression of a cDNA encoding the membrane-associated rat intestinal alkaline phosphatase Biochimica et biophysica acta, 1037 2, 170Lowe,M.E.; Rosenblum,J.L.; McEwen,P.; Strauss,A.W. 1990. Cloning and characterization of the human colipase cDNA Biochemistry, 29 3, 823Lowe,M.E.; Strauss,A.W. 1990. Expression of a Nagao-type, phosphatidylinositol-glycan anchored alkaline phosphatase in human choriocarcinomas Cancer research, 50 13, 3956Strauss,A.W.; Duran,M.; Zhang,Z.F.; Alpers,R.; Kelly,D.P. 1990. Molecular analysis of medium chain acyl-CoA dehydrogenase deficiency Progress in clinical and biological research, 321 , 609Strauss,A.W.; Goldring,D. 1990. Coarctation of the aorta in infants Circulation, 82 5, 1882Billadello,J.J.; Fontanet,H.L.; Strauss,A.W.; Abendschein,D.R. 1989. Characterization of MB creatine kinase isoform conversion in vitro and in vivo in dogs The Journal of clinical investigation, 83 5, 1637Chu,T.W.; Eftime,R.; Sztul,E.; Strauss,A.W. 1989. Synthetic transit peptides inhibit import and processing of mitochondrial precursor proteins The Journal of biological chemistry, 264 16, 9552Gast,M.J.; Sims,H.F.; Murdock,G.L.; Gast,P.M.; Strauss,A.W. 1989. Isolation and sequencing of a complementary deoxyribonucleic acid clone encoding human placental 17 beta-estradiol dehydrogenase: identification of the putative cofactor binding site American Journal of Obstetrics and Gynecology, 161 6 Pt 1, 1726Haas,R.C.; Korenfeld,C.; Zhang,Z.F.; Perryman,B.; Roman,D.; Strauss,A.W. 1989. Isolation and characterization of the gene and cDNA encoding human mitochondrial creatine kinase The Journal of biological chemistry, 264 5, 2890Kelly,D.P.; Gordon,J.I.; Alpers,R.; Strauss,A.W. 1989. The tissue-specific expression and developmental regulation of two nuclear genes encoding rat mitochondrial proteins. Medium chain acyl-CoA dehydrogenase and mitochondrial malate dehydrogenase The Journal of biological chemistry, 264 32, 18921Lowe,M.E.; Rosenblum,J.L.; Strauss,A.W. 1989. Cloning and characterization of human pancreatic lipase cDNA The Journal of biological chemistry, 264 33, 20042Sztul,E.S.; Chu,T.W.; Strauss,A.W.; Rosenberg,L.E. 1989. Translocation of precursor proteins into the mitochondrial matrix occurs through an environment accessible to aqueous perturbants Journal of cell science, 94 ( Pt 4) Pt 4, 695Griffin,M.L.; Hernandez,A.; Martin,T.C.; Goldring,D.; Bolman,R.M.; Spray,T.L.; Strauss,A.W. 1988. Dilated cardiomyopathy in infants and children Journal of the American College of Cardiology, 11 1, 139Stallings,R.L.; Olson,E.; Strauss,A.W.; Thompson,L.H.; Bachinski,L.L.; Siciliano,M.J. 1988. Human creatine kinase genes on chromosomes 15 and 19, and proximity of the gene for the muscle form to the genes for apolipoprotein C2 and excision repair American Journal of Human Genetics, 43 2, 144Sztul,E.S.; Chu,T.W.; Strauss,A.W.; Rosenberg,L.E. 1988. Import of the malate dehydrogenase precursor by mitochondria. Cleavage within leader peptide by matrix protease leads to formation of intermediate-sized form The Journal of biological chemistry, 263 24, 12085Trask,R.V.; Strauss,A.W.; Billadello,J.J. 1988. Developmental regulation and tissue-specific expression of the human muscle creatine kinase gene The Journal of biological chemistry, 263 32, 17142Abendschein,D.R.; Serota,H.; Plummer,T.H.,Jr; Amiraian,K.; Strauss,A.W.; Sobel,B.E.; Jaffe,A.S. 1987. Conversion of MM creatine kinase isoforms in human plasma by carboxypeptidase N The Journal of laboratory and clinical medicine, 110 6, 798Chu,T.W.; Grant,P.M.; Strauss,A.W. 1987. Mutation of a neutral amino acid in the transit peptide of rat mitochondrial malate dehydrogenase abolishes binding and import The Journal of biological chemistry, 262 32, 15759Chu,T.W.; Grant,P.M.; Strauss,A.W. 1987. The role of arginine residues in the rat mitochondrial malate dehydrogenase transit peptide The Journal of biological chemistry, 262 26, 12806Grace,A.M.; Gualdoni,J.; Strauss,A.W.; Sobel,B.E. 1987. Quantification of isoforms of plasma MM creatine kinase (CK) with an immunoblot procedure Catheterization and cardiovascular diagnosis, 13 1, 26Grant,P.M.; Roderick,S.L.; Grant,G.A.; Banaszak,L.J.; Strauss,A.W. 1987. Comparison of the precursor and mature forms of rat heart mitochondrial malate dehydrogenase Biochemistry, 26 1, 128Hortin,G.; Fok,K.F.; Toren,P.C.; Strauss,A.W. 1987. Sulfation of a tyrosine residue in the plasmin-binding domain of alpha 2-antiplasmin The Journal of biological chemistry, 262 7, 3082Kelly,D.P.; Kim,J.J.; Billadello,J.J.; Hainline,B.E.; Chu,T.W.; Strauss,A.W. 1987. Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue Proceedings of the National Academy of Sciences of the United States of America, 84 12, 4068Seetharam,S.; Ovitt,C.; Strauss,A.W.; Rubin,D.; Alpers,D.H. 1987. Fat feeding stimulates only one of the two mRNAs encoding rat intestinal membranous and secreted alkaline phosphatase Biochemical and biophysical research communications, 145 1, 363Alpers,D.H.; Helms,D.; Seetharam,S.; May,V.L.; Strauss,A.W. 1986. In vitro translation of intestinal sucrase-isomaltase and glucoamylase Biochemical and biophysical research communications, 134 1, 37Billadello,J.J.; Kelly,D.P.; Roman,D.G.; Strauss,A.W. 1986. The complete nucleotide sequence of canine brain B creatine kinase mRNA: homology in the coding and 3' noncoding regions among species Biochemical and biophysical research communications, 138 1, 392Canter,C.E.; Martin,T.C.; Spray,T.L.; Weldon,C.S.; Strauss,A.W. 1986. Scimitar syndrome in childhood The American Journal of Cardiology, 58 7, 652Gordon,J.I.; Sims,H.F.; Strauss,A.W.; Scanu,A.M.; Edelstein,C.; Byrne,R.E. 1986. Proteolytic processing and compartmentalization of the primary translation products of mammalian apolipoprotein mRNAs CRC critical reviews in biochemistry, 20 1, 37Grant,P.M.; Tellam,J.; May,V.L.; Strauss,A.W. 1986. Isolation and nucleotide sequence of a cDNA clone encoding rat mitochondrial malate dehydrogenase Nucleic acids research, 14 15, 6053Hortin,G.; Chan,A.C.; Fok,K.F.; Strauss,A.W.; Atkinson,J.P. 1986. Sequence analysis of the COOH terminus of the alpha-chain of the fourth component of human complement. Identification of the site of its extracellular cleavage The Journal of biological chemistry, 261 19, 9065Hortin,G.; Folz,R.; Gordon,J.I.; Strauss,A.W. 1986. Characterization of sites of tyrosine sulfation in proteins and criteria for predicting their occurrence Biochemical and biophysical research communications, 141 1, 326Hortin,G.; Green,E.D.; Baenziger,J.U.; Strauss,A.W. 1986. Sulphation of proteins secreted by a human hepatoma-derived cell line. Sulphation of N-linked oligosaccharides on alpha 2HS-glycoprotein The Biochemical journal, 235 2, 407Hortin,G.; Sims,H.; Strauss,A.W. 1986. Identification of the site of sulfation of the fourth component of human complement The Journal of biological chemistry, 261 4, 1786Hortin,G.; Strauss,A.W. 1986. Effects of acidotropic compounds on the secretory pathway: inhibition of secretion and processing of the third and fourth components of complement Biochemical and biophysical research communications, 136 2, 603Hortin,G.; Tollefsen,D.M.; Strauss,A.W. 1986. Identification of two sites of sulfation of human heparin cofactor II The Journal of biological chemistry, 261 34, 15827Ovitt,C.E.; Strauss,A.W.; Alpers,D.H.; Chou,J.Y.; Boime,I. 1986. Expression of different-sized placental alkaline phosphatase mRNAs in placenta and choriocarcinoma cells Proceedings of the National Academy of Sciences of the United States of America, 83 11, 3781Billadello,J.J.; Roman,D.G.; Grace,A.M.; Sobel,B.E.; Strauss,A.W. 1985. The nature of post-translational formation of MM creatine kinase isoforms The Journal of biological chemistry, 260 28, 14988Gordon,J.I.; Sims,H.F.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1985. Extracellular processing of proapolipoprotein A-II in Hep G2 cell cultures is mediated by a 54-kDa protease immunologically related to cathepsin B The Journal of biological chemistry, 260 27, 14824Grace,A.M.; Strauss,A.W.; Sobel,B.E. 1985. Sensitive quantification of isoforms of canine MM creatine kinase with an immunoblot procedure suitable for large numbers of samples Analytical Biochemistry, 149 1, 209Hashimoto,H.; Abendschein,D.R.; Strauss,A.W.; Sobel,B.E. 1985. Early detection of myocardial infarction in conscious dogs by analysis of plasma MM creatine kinase isoforms Circulation, 71 2, 363Alpers,D.H.; Strauss,A.W.; Ockner,R.K.; Bass,N.M.; Gordon,J.I. 1984. Cloning of a cDNA encoding rat intestinal fatty acid binding protein Proceedings of the National Academy of Sciences of the United States of America, 81 2, 313Blaufuss,M.C.; Gordon,J.I.; Schonfeld,G.; Strauss,A.W.; Alpers,D.H. 1984. Biosynthesis of apolipoprotein C-III in rat liver and small intestinal mucosa The Journal of biological chemistry, 259 4, 2452Gordon,J.I.; Bisgaier,C.L.; Sims,H.F.; Sachdev,O.P.; Glickman,R.M.; Strauss,A.W. 1984. Biosynthesis of human preapolipoprotein A-IV The Journal of biological chemistry, 259 1, 468Gordon,J.I.; Sims,H.F.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1984. Human proapolipoprotein A-II is cleaved following secretion from Hep G2 cells by a thiol protease The Journal of biological chemistry, 259 24, 15556Hashimoto,H.; Grace,A.M.; Billadello,J.J.; Gross,R.W.; Strauss,A.W.; Sobel,B.E. 1984. Nondenaturing quantification of subforms of canine MM creatine kinase isoenzymes (isoforms) and their interconversion The Journal of laboratory and clinical medicine, 103 3, 470Leonard,W.J.; Strauss,A.W.; Go,M.F.; Alpers,D.H.; Gordon,J.I. 1984. Biosynthesis and compartmentalization of rat-intestinal vitamin-D-dependent calcium-binding protein European journal of biochemistry / FEBS, 139 3, 561Lowe,J.B.; Strauss,A.W.; Gordon,J.I. 1984. Expression of a mammalian fatty acid-binding protein in Escherichia coli The Journal of biological chemistry, 259 20, 12696Cockerham,J.T.; Martin,T.C.; Gutierrez,F.R.; Hartmann,A.F.,Jr; Goldring,D.; Strauss,A.W. 1983. Spontaneous closure of secundum atrial septal defect in infants and young children The American Journal of Cardiology, 52 10, 1267Edelstein,C.; Gordon,J.I.; Toscas,K.; Sims,H.F.; Strauss,A.W.; Scanu,A.M. 1983. In vitro conversion of proapoprotein A-I to apoprotein A-I. Partial characterization of an extracellular enzyme activity The Journal of biological chemistry, 258 19, 11430Gordon,J.I.; Alpers,D.H.; Ockner,R.K.; Strauss,A.W. 1983. The nucleotide sequence of rat liver fatty acid binding protein mRNA The Journal of biological chemistry, 258 5, 3356Gordon,J.I.; Budelier,K.A.; Sims,H.F.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1983. Biosynthesis of human preproapolipoprotein A-II The Journal of biological chemistry, 258 22, 14054Gordon,J.I.; Sims,H.F.; Lentz,S.R.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1983. Proteolytic processing of human preproapolipoprotein A-I. A proposed defect in the conversion of pro A-I to A-I in Tangier's disease The Journal of biological chemistry, 258 6, 4037Perryman,M.B.; Strauss,A.W.; Buettner,T.L.; Roberts,R. 1983. Molecular heterogeneity of creatine kinase isoenzymes Biochimica et biophysica acta, 747 3, 284Perryman,M.B.; Strauss,A.W.; Olson,J.; Roberts,R. 1983. In vitro translation of canine mitochondrial creatine kinase messenger RNA Biochemical and biophysical research communications, 110 3, 967Gordon,J.I.; Smith,D.P.; Alpers,D.H.; Strauss,A.W. 1982. Cloning of a complementary deoxyribonucleic acid encoding a portion of rat intestinal preapolipoprotein AIV messenger ribonucleic acid Biochemistry, 21 22, 5424Gordon,J.I.; Smith,D.P.; Alpers,D.H.; Strauss,A.W. 1982. Proteolytic processing of the primary translation product of rat intestinal apolipoprotein A-IV mRNA. Comparison with preproapolipoprotein A-I processing The Journal of biological chemistry, 257 14, 8418Gordon,J.I.; Smith,D.P.; Andy,R.; Alpers,D.H.; Schonfeld,G.; Strauss,A.W. 1982. The primary translation product of rat intestinal apolipoprotein A-I mRNA is an unusual preproprotein The Journal of biological chemistry, 257 2, 971Graves,C.B.; Munns,T.W.; Willingham,A.K.; Strauss,A.W. 1982. Rat factor X is synthesized as a single chain precursor inducible by prothrombin fragments The Journal of biological chemistry, 257 21, 13108Strauss,A.W.; Boime,I. 1982. Compartmentation of newly synthesized proteins CRC critical reviews in biochemistry, 12 3, 205Taysi,K.; Strauss,A.W.; Yang,V.; Padmalatha,C.; Marshall,R.E. 1982. Terminal deletion of the long arm of chromosome 10 : q26 to qter. Case report and review of literature Annales de Genetique, 25 3, 141Canter,C.E.; Bower,R.J.; Strauss,A.W. 1981. Atypical Kawasaki disease with aortic aneurysm Pediatrics, 68 6, 885Graves,C.B.; Munns,T.W.; Carlisle,T.L.; Grant,G.A.; Strauss,A.W. 1981. Induction of prothrombin synthesis by prothrombin fragments Proceedings of the National Academy of Sciences of the United States of America, 78 8, 4772Henry,C.G.; Goldring,D.; Hartmann,A.F.; Weldon,C.S.; Strauss,A.W. 1981. Treatment of d-transposition of the great arteries: management of hypoxemia after balloon atrial septostomy The American Journal of Cardiology, 47 2, 299Henry,C.G.; Gutierrez,F.; Lee,J.T.; Hartmann,A.F.; Bell,M.J.; Bower,R.J.; Strauss,A.W. 1981. Aortic thrombosis presenting as congestive heart failure: an umbilical artery catheter complication The Journal of pediatrics, 98 5, 820Henry,C.G.; Strauss,A.W.; Keating,J.P.; Hillman,R.E. 1981. Congestive cardiomyopathy associated with beta-ketothiolase deficiency The Journal of pediatrics, 99 5, 754Mumford,R.A.; Pickett,C.B.; Zimmerman,M.; Strauss,A.W. 1981. Protease activities present in wheat germ and rabbit reticulocyte lysates Biochemical and biophysical research communications, 103 2, 565Mumford,R.A.; Pierzchala,P.A.; Strauss,A.W.; Zimmerman,M. 1981. Purification of a membrane-bound metalloendopeptidase from porcine kidney that degrades peptide hormones Proceedings of the National Academy of Sciences of the United States of America, 78 11, 6623Mumford,R.A.; Strauss,A.W.; Powers,J.C.; Pierzchala,P.A.; Nishino,N.; Zimmerman,M. 1980. A zinc metalloendopeptidase associated with dog pancreatic membranes The Journal of biological chemistry, 255 6, 2227Strauss,A.W.; Zimmerman,M.; Mumford,R.A.; Alberts,A.W. 1980. Processing of pre-proalbumin and pre-placental lactogen Annals of the New York Academy of Sciences, 343 , 168Zimmerman,M.; Ashe,B.M.; Alberts,A.W.; Pierzchala,P.A.; Powers,J.C.; Nishino,N.; Strauss,A.W.; Mumford,R.A. 1980. Protease activities present in dog pancreatic membranes that process human pre-placental lactogen Annals of the New York Academy of Sciences, 343 , 405Haymond,M.W.; Strauss,A.W.; Arnold,K.J.; Bier,D.M. 1979. Glucose homeostasis in children with severe cyanotic congenital heart disease The Journal of pediatrics, 95 2, 220Jones,L.A.; Jordan,D.K.; Taysi,K.; Strauss,A.W.; Toth,J.K. 1979. Partial duplication of the long arm of chromosome 5: a case due to balanced paternal translocation and review of the literature Human genetics, 51 1, 37Jones,L.A.; Taysi,K.; Strauss,A.W.; Hartmann,A.F. 1979. Partial trisomy 13 as a result of de novo (6p;13q) translocation Human genetics, 48 2, 245Strauss,A.W.; Zimmerman,M.; Boime,I.; Ashe,B.; Mumford,R.A.; Alberts,A.W. 1979. Characterization of an endopeptidase involved in pre-protein processing Proceedings of the National Academy of Sciences of the United States of America, 76 9, 4225Taysi,K.; Noetzel,M.J.; Strauss,A.W. 1979. Presumptive long arm deletion of chromosome 8: a new syndrome? Human genetics, 51 1, 49Hernandez,A.; Strauss,A.W.; McKnight,R.; Hartmann,A.F.,Jr. 1978. Diagnosis of pulmonary arteriovenous fistula by contrast echocardiography The Journal of pediatrics, 93 2, 258Strauss,A.W.; Bennett,C.A.; Donohue,A.M.; Rodkey,J.A.; Boime,I.; Alberts,A.W. 1978. Conversion of rat pre-proalbumin to proalbumin in vitro by ascites membranes. Demonstration by NH2-TERMINAL SEQUENCE ANALYSIS The Journal of biological chemistry, 253 17, 6270Strauss,A.W.; Bennett,C.D.; Donohue,A.M.; Rodkey,J.A.; Alberts,A.W. 1977. Rat liver pre-proalbumin: complete amino acid sequence of the pre-piece. Analysis of the direct translation product of albumin messenger RNA The Journal of biological chemistry, 252 19, 6846Strauss,A.W.; Donohue,A.M.; Bennett,C.D.; Rodkey,J.A.; Alberts,A.W. 1977. Rat liver preproalbumin: in vitro synthesis and partial amino acid sequence Proceedings of the National Academy of Sciences of the United States of America, 74 4, 1358Alberts,A.W.; Strauss,A.W.; Hennessy,S.; Vagelos,P.R. 1975. Regulation of synthesis of hepatic fatty acid synthetase: binding of fatty acid synthetase antibodies to polysomes Proceedings of the National Academy of Sciences of the United States of America, 72 10, 3956Alderson,P.O.; Jost,R.G.; Strauss,A.W.; Boonvisut,S.; Markham,J. 1975. Radionuclide angiocardiography. Improved diagnosis and quantitation of left-to-right shunts using area ratio techniques in children Circulation, 51 6, 1136Strauss,A.W.; Alberts,A.W.; Hennessy,S.; Vagelos,P.R. 1975. Regulation of synthesis of hepatic fatty acid synthetase: polysomal translation in a cell-free system Proceedings of the National Academy of Sciences of the United States of America, 72 11, 4366Strauss,A.W.; Santa-Maria,M.; Goldring,D. 1975. Constrictive pericarditis in children American Journal of Diseases of Children (1960), 129 7, 822Strauss,A.W.; Escobedo,M.; Goldring,D. 1974. Continuous monitoring of arterial oxygen tension in the newborn infant The Journal of pediatrics, 85 2, 254Strauss,A.W.; Goldring,D. 1974. Editorial: Valve replacement in acute rheumatic heart disease The Journal of pediatrics, 84 5, 786Strauss,A.W.; Goldring,D.; Kissane,J.; Hernandez,A.; Hartmann,A.F.; McKnight,C.R.; Weldon,C.S. 1974. Valve replacement in acute rheumatic heart disease The Journal of thoracic and cardiovascular surgery, 67 4, 659Keating,J.P.; Lell,M.E.; Strauss,A.W.; Zarkowsky,H.; Smith,G.E. 1973. Infantile methemoglobinemia caused by carrot juice The New England journal of medicine, 288 16, 824STRAUSS,A.W.; STRAUSS,A.W.,Jr. 1952. Pseudomucinous cystadenocarcinoma of the ovary American Journal of Obstetrics and Gynecology, 64 6, 1382Bennett, Michael J; Russell, Laurie K; Tokunaga, Chonan; Narayan, Srinivas B; Tan, Lu; Seegmiller, Adam; Boriack, Richard L; Strauss, Arnold W . Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase. Molecular genetics and metabolism, 89 1-2, 74-9
Peer Reviewed Publications Acehan, Devrim; Vaz, Frederic; Houtkooper, Riekelt H; James, Jeanne; Moore, Vicky; Tokunaga, Chonan; Kulik, Willem; Wansapura, Janaka; Toth, Matthew J; Strauss, Arnold; Khuchua, Zaza 2011. Cardiac and skeletal muscle defects in a mouse model of human Barth syndrome. The Journal of biological chemistry, 286 2, 899-908Britigan, Bradley; Strauss, Arnold; Susman, Jeff 2010. Salami science or editorial imperialism? The Journal of pediatrics, 157 3, 518-9; author reply Li, Changhong; Chen, Pan; Palladino, Andrew; Narayan, Srinivas; Russell, Laurie K; Sayed, Samir; Xiong, Guoxiang; Chen, Jie; Stokes, David; Butt, Yasmeen M; Jones, Patricia M; Collins, Heather W; Cohen, Noam A; Cohen, Akiva S; Nissim, Itzhak; Smith, Thomas J; Strauss, Arnold W; Matschinsky, Franz M; Bennett, Michael J; Stanley, Charles A 2010. Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase. The Journal of biological chemistry, 285 41, 31806-18Arnold, Georgianne L; Van Hove, Johan; Freedenberg, Debra; Strauss, Arnold; Longo, Nicola; Burton, Barbara; Garganta, Cheryl; Ficicioglu, Can; Cederbaum, Stephen; Harding, Cary; Boles, Richard G; Matern, Dietrich; Chakraborty, Pranesh; Feigenbaum, Annette 2009. A Delphi clinical practice protocol for the management of very long chain acyl-CoA dehydrogenase deficiency. Molecular genetics and metabolism, 96 3, 85-90Chappell, Tresa; Creech, C Buddy; Parra, David; Strauss, Arnold; Scholl, Frank; Whitney, Gina 2008. Presentation of pulmonary artery intimal sarcoma in an infant with a history of neonatal valvular pulmonic stenosis. The Annals of thoracic surgery, 85 3, 1092-4Hsu, Ho-Wen; Zytkovicz, Thomas H; Comeau, Anne Marie; Strauss, Arnold W; Marsden, Deborah; Shih, Vivian E; Grady, George F; Eaton, Roger B 2008. Spectrum of medium-chain acyl-CoA dehydrogenase deficiency detected by newborn screening. Pediatrics, 121 5, e1108-14Werdich, Andreas A; Baudenbacher, Franz; Dzhura, Igor; Jeyakumar, Loice H; Kannankeril, Prince J; Fleischer, Sidney; LeGrone, Alison; Milatovic, Dejan; Aschner, Michael; Strauss, Arnold W; Anderson, Mark E; Exil, Vernat J 2007. Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice. American journal of physiology. Heart and circulatory physiology, 292 5, H2202-11Werdich,A.A.; Baudenbacher,F.; Dzhura,I.; Jeyakumar,L.H.; Kannankeril,P.J.; Fleischer,S.; Legrone,A.; Milatovic,D.; Aschner,M.; Strauss,A.W.; Anderson,M.; Exil,V.J. 2007. Polymorphic Ventricular Tachycardia and Abnormal Calcium Handling in Very-Long-Chain Acyl-CoA Dehydrogenase Null Mice American Journal of Physiology. Heart and Circulatory Physiology, , Bennett,M.J.; Russell,L.K.; Tokunaga,C.; Narayan,S.B.; Tan,L.; Seegmiller,A.; Boriack,R.L.; Strauss,A.W. 2006. Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase Molecular genetics and metabolism, 89 1-2, 74Djouadi,F.; Aubey,F.; Schlemmer,D.; Gobin,S.; Laforet,P.; Wanders,R.J.; Strauss,A.W.; Bonnefont,J.P.; Bastin,J. 2006. Potential of fibrates in the treatment of fatty acid oxidation disorders: revival of classical drugs? Journal of inherited metabolic disease, 29 2-3, 341Exil, Vernat J; Gardner, Carla D; Rottman, Jeffrey N; Sims, Harold; Bartelds, Beatrijs; Khuchua, Zaza; Sindhal, Rekha; Ni, Gemin; Strauss, Arnold W 2006. Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase. American journal of physiology. Heart and circulatory physiology, 290 3, H1289-97Exil,V.J.; Gardner,C.D.; Rottman,J.N.; Sims,H.; Bartelds,B.; Khuchua,Z.; Sindhal,R.; Ni,G.; Strauss,A.W. 2006. Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase American journal of physiology.Heart and circulatory physiology, 290 3, H1289Hoffman, Jodi D; Steiner, Robert D; Paradise, Lori; Harding, Carey O; Ding, Li; Strauss, Arnold W; Kaplan, Paige 2006. Rhabdomyolysis in the military: recognizing late-onset very long-chain acyl Co-A dehydrogenase deficiency. Military medicine, 171 7, 657-8Hoffman,J.D.; Steiner,R.D.; Paradise,L.; Harding,C.O.; Ding,L.; Strauss,A.W.; Kaplan,P. 2006. Rhabdomyolysis in the military: recognizing late-onset very long-chain acyl Co-A dehydrogenase deficiency Military medicine, 171 7, 657Khuchua, Zaza; Yue, Zou; Batts, Lorene; Strauss, Arnold W 2006. A zebrafish model of human Barth syndrome reveals the essential role of tafazzin in cardiac development and function. Circulation research, 99 2, 201-8Khuchua,Z.; Yue,Z.; Batts,L.; Strauss,A.W. 2006. A zebrafish model of human Barth syndrome reveals the essential role of tafazzin in cardiac development and function Circulation research, 99 2, 201Liebig, Michaela; Schymik, Ina; Mueller, Martina; Wendel, Udo; Mayatepek, Ertan; Ruiter, Jos; Strauss, Arnold W; Wanders, Ronald J A; Spiekerkoetter, Ute 2006. Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels. Pediatrics, 118 3, 1065-9Liebig,M.; Gyenes,M.; Brauers,G.; Ruiter,J.P.; Wendel,U.; Mayatepek,E.; Strauss,A.W.; Wanders,R.J.; Spiekerkoetter,U. 2006. Carnitine supplementation induces long-chain acylcarnitine production--studies in the VLCAD-deficient mouse Journal of inherited metabolic disease, 29 2-3, 343Liebig,M.; Schymik,I.; Mueller,M.; Wendel,U.; Mayatepek,E.; Ruiter,J.; Strauss,A.W.; Wanders,R.J.; Spiekerkoetter,U. 2006. Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels Pediatrics, 118 3, 1065Liske, Michael R; Greeley, Christopher S; Law, David J; Reich, Jonathan D; Morrow, William R; Baldwin, H Scott; Graham, Thomas P; Strauss, Arnold W; Kavanaugh-McHugh, Ann L; Walsh, William F 2006. Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease. Pediatrics, 118 4, e1250-6Liske,M.R.; Greeley,C.S.; Law,D.J.; Reich,J.D.; Morrow,W.R.; Baldwin,H.S.; Graham,T.P.; Strauss,A.W.; Kavanaugh-McHugh,A.L.; Walsh,W.F.; Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease. 2006. Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease Pediatrics, 118 4, e1250Schymik, Ina; Liebig, Michaela; Mueller, Martina; Wendel, Udo; Mayatepek, Ertan; Strauss, Arnold W; Wanders, Ronald J A; Spiekerkoetter, Ute 2006. Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry. The Journal of pediatrics, 149 1, 128-30Schymik,I.; Liebig,M.; Mueller,M.; Wendel,U.; Mayatepek,E.; Strauss,A.W.; Wanders,R.J.; Spiekerkoetter,U. 2006. Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry The Journal of pediatrics, 149 1, 128Spiekerkoetter,U.; Ruiter,J.; Tokunaga,C.; Wendel,U.; Mayatepek,E.; Wijburg,F.A.; Strauss,A.W.; Wanders,R.J. 2006. Evidence for impaired gluconeogenesis in very long-chain acyl-CoA dehydrogenase-deficient mice Hormone and metabolic research.Hormon- und Stoffwechselforschung.Hormones et metabolisme, 38 10, 625Djouadi,F.; Aubey,F.; Schlemmer,D.; Ruiter,J.P.; Wanders,R.J.; Strauss,A.W.; Bastin,J. 2005. Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders Human molecular genetics, 14 18, 2695Shekhawat, Prem S; Matern, Dietrich; Strauss, Arnold W 2005. Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and management. Pediatric research, 57 5 Pt 2, 78R-86RShekhawat,P.S.; Matern,D.; Strauss,A.W. 2005. Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and management Pediatric research, 57 5 Pt 2, 78RSpiekerkoetter, Ute; Tokunaga, Chonan; Wendel, Udo; Mayatepek, Ertan; Ijlst, Lodewijk; Vaz, Frederic M; van Vlies, Naomi; Overmars, Henk; Duran, Marinus; Wijburg, Frits A; Wanders, Ronald J; Strauss, Arnold W 2005. Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. Pediatric research, 57 6, 760-4Spiekerkoetter,U.; Tokunaga,C.; Wendel,U.; Mayatepek,E.; Ijlst,L.; Vaz,F.M.; van Vlies,N.; Overmars,H.; Duran,M.; Wijburg,F.A.; Wanders,R.J.; Strauss,A.W. 2005. Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice Pediatric research, 57 6, 760Strauss, Arnold W 2005. Surprising? Perhaps not. Long-chain fatty acid oxidation during human fetal development. Pediatric research, 57 6, 753-4Strauss,A.W. 2005. Surprising? Perhaps not. Long-chain fatty acid oxidation during human fetal development Pediatric research, 57 6, 753McKinney,J.T.; Longo,N.; Hahn,S.H.; Matern,D.; Rinaldo,P.; Strauss,A.W.; Dobrowolski,S.F. 2004. Rapid, comprehensive screening of the human medium chain acyl-CoA dehydrogenase gene Molecular genetics and metabolism, 82 2, 112Spiekerkoetter, Ute; Bennett, Michael J; Ben-Zeev, Bruria; Strauss, Arnold W; Tein, Ingrid 2004. Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein. Muscle & nerve, 29 1, 66-72Spiekerkoetter, Ute; Khuchua, Zaza; Yue, Zou; Bennett, Michael J; Strauss, Arnold W 2004. General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnover. Pediatric research, 55 2, 190-6Spiekerkoetter,U.; Bennett,M.J.; Ben-Zeev,B.; Strauss,A.W.; Tein,I. 2004. Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein Muscle & nerve, 29 1, 66Spiekerkoetter,U.; Khuchua,Z.; Yue,Z.; Bennett,M.J.; Strauss,A.W. 2004. General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnover Pediatric research, 55 2, 190Spiekerkoetter,U.; Tokunaga,C.; Wendel,U.; Mayatepek,E.; Exil,V.; Duran,M.; Wijburg,F.A.; Wanders,R.J.; Strauss,A.W. 2004. Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress European journal of clinical investigation, 34 3, 191Spierkerkoetter,U.; Khuchua,Z.; Yue,Z.; Strauss,A.W. 2004. The early-onset phenotype of mitochondrial trifunctional protein deficiency: a lethal disorder with multiple tissue involvement Journal of inherited metabolic disease, 27 2, 294Strauss, Arnold W 2004. Tandem mass spectrometry in discovery of disorders of the metabolome. The Journal of clinical investigation, 113 3, 354-6Strauss,A.W. 2004. Tandem mass spectrometry in discovery of disorders of the metabolome The Journal of clinical investigation, 113 3, 354Tsirka, Anna E; Trinkaus, Kathryn; Chen, Su-Chiung; Lipshultz, Steven E; Towbin, Jeffrey A; Colan, Steven D; Exil, Vernat; Strauss, Arnold W; Canter, Charles E 2004. Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation. Journal of the American College of Cardiology, 44 2, 391-7Tsirka,A.E.; Trinkaus,K.; Chen,S.C.; Lipshultz,S.E.; Towbin,J.A.; Colan,S.D.; Exil,V.; Strauss,A.W.; Canter,C.E. 2004. Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation Journal of the American College of Cardiology, 44 2, 391Tyni, Tiina; Paetau, Anders; Strauss, Arnold W; Middleton, Bruce; Kivelä, Tero 2004. Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Pediatric research, 56 5, 744-50Tyni,T.; Paetau,A.; Strauss,A.W.; Middleton,B.; Kivela,T. 2004. Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency Pediatric research, 56 5, 744Boero,J.; Qin,W.; Cheng,J.; Woolsey,T.A.; Strauss,A.W.; Khuchua,Z. 2003. Restricted neuronal expression of ubiquitous mitochondrial creatine kinase: changing patterns in development and with increased activity Molecular and cellular biochemistry, 244 1-2, 69Exil, Vernat J; Roberts, Richard L; Sims, Harold; McLaughlin, Jacquelin E; Malkin, Robert A; Gardner, Carla D; Ni, Gemin; Rottman, Jeffrey N; Strauss, Arnold W 2003. Very-long-chain acyl-coenzyme a dehydrogenase deficiency in mice. Circulation research, 93 5, 448-55Exil,V.J.; Roberts,R.L.; Sims,H.; McLaughlin,J.E.; Malkin,R.A.; Gardner,C.D.; Ni,G.; Rottman,J.N.; Strauss,A.W. 2003. Very-long-chain acyl-coenzyme a dehydrogenase deficiency in mice Circulation research, 93 5, 448Khuchua,Z.; Wozniak,D.F.; Bardgett,M.E.; Yue,Z.; McDonald,M.; Boero,J.; Hartman,R.E.; Sims,H.; Strauss,A.W. 2003. Deletion of the N-terminus of murine map2 by gene targeting disrupts hippocampal ca1 neuron architecture and alters contextual memory Neuroscience, 119 1, 101Lee,J.E.; Yoon,H.R.; Paik,K.H.; Hwang,S.J.; Shim,J.W.; Chang,Y.S.; Park,W.S.; Strauss,A.W.; Jin,D.K. 2003. A case of mitochondrial trifunctional protein deficiency diagnosed by acylcarnitine profile and DNA analysis in a dried blood spot of a 4-day-old boy Journal of inherited metabolic disease, 26 4, 403Shekhawat, Prem; Bennett, Michael J; Sadovsky, Yoel; Nelson, D Michael; Rakheja, Dinesh; Strauss, Arnold W 2003. Human placenta metabolizes fatty acids: implications for fetal fatty acid oxidation disorders and maternal liver diseases. American journal of physiology. Endocrinology and metabolism, 284 6, E1098-105Shekhawat,P.; Bennett,M.J.; Sadovsky,Y.; Nelson,D.M.; Rakheja,D.; Strauss,A.W. 2003. Human placenta metabolizes fatty acids: implications for fetal fatty acid oxidation disorders and maternal liver diseases American journal of physiology.Endocrinology and metabolism, 284 6, E1098Spiekerkoetter, Ute; Sun, Bin; Khuchua, Zaza; Bennett, Michael J; Strauss, Arnold W 2003. Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations. Human mutation, 21 6, 598-607Spiekerkoetter, Ute; Sun, Bin; Zytkovicz, Thomas; Wanders, Ronald; Strauss, Arnold W; Wendel, Udo 2003. MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency. The Journal of pediatrics, 143 3, 335-42Spiekerkoetter,U.; Sun,B.; Khuchua,Z.; Bennett,M.J.; Strauss,A.W. 2003. Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations Human mutation, 21 6, 598Spiekerkoetter,U.; Sun,B.; Zytkovicz,T.; Wanders,R.; Strauss,A.W.; Wendel,U. 2003. MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency The Journal of pediatrics, 143 3, 335Strauss, Arnold; Lock, James E 2003. Pediatric cardiomyopathy--a long way to go. The New England journal of medicine, 348 17, 1703-5Hintz, Susan R; Matern, Dietrich; Strauss, Arnold; Bennett, Michael J; Hoyme, H Eugene; Schelley, Susan; Kobori, Joyce; Colby, Christopher; Lehman, Norman L; Enns, Gregory M 2002. Early neonatal diagnosis of long-chain 3-hydroxyacyl coenzyme a dehydrogenase and mitochondrial trifunctional protein deficiencies. Molecular genetics and metabolism, 75 2, 120-7Spiekerkoetter, Ute; Eeds, Angela; Yue, Zou; Haines, Jonathan; Strauss, Arnold W; Summar, Marshall 2002. Uniparental disomy of chromosome 2 resulting in lethal trifunctional protein deficiency due to homozygous alpha-subunit mutations. Human mutation, 20 6, 447-51Spiekerkoetter,U.; Eeds,A.; Yue,Z.; Haines,J.; Strauss,A.W.; Summar,M. 2002. Uniparental disomy of chromosome 2 resulting in lethal trifunctional protein deficiency due to homozygous alpha-subunit mutations Human mutation, 20 6, 447Straussberg, Rachel; Strauss, Arnold W 2002. A novel mutation of late-onset very-long-chain acyl-CoA dehydrogenase deficiency. Pediatric neurology, 27 2, 136-7Straussberg,R.; Strauss,A.W. 2002. A novel mutation of late-onset very-long-chain acyl-CoA dehydrogenase deficiency Pediatric neurology, 27 2, 136Yang, Zi; Yamada, Jennifer; Zhao, Yiwen; Strauss, Arnold W; Ibdah, Jamal A 2002. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease. JAMA : the journal of the American Medical Association, 288 17, 2163-6Yang, Zi; Zhao, Yiwen; Bennett, Michael J; Strauss, Arnold W; Ibdah, Jamal A 2002. Fetal genotypes and pregnancy outcomes in 35 families with mitochondrial trifunctional protein mutations. American journal of obstetrics and gynecology, 187 3, 715-20Yang,Z.; Yamada,J.; Zhao,Y.; Strauss,A.W.; Ibdah,J.A. 2002. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease JAMA : the journal of the American Medical Association, 288 17, 2163Yang,Z.; Zhao,Y.; Bennett,M.J.; Strauss,A.W.; Ibdah,J.A. 2002. Fetal genotypes and pregnancy outcomes in 35 families with mitochondrial trifunctional protein mutations American Journal of Obstetrics and Gynecology, 187 3, 715Albers,S.; Levy,H.L.; Irons,M.; Strauss,A.W.; Marsden,D. 2001. Compound heterozygosity in four asymptomatic siblings with medium-chain acyl-CoA dehydrogenase deficiency Journal of inherited metabolic disease, 24 3, 417Barycki,J.J.; O'Brien,L.K.; Strauss,A.W.; Banaszak,L.J. 2001. Glutamate 170 of human l-3-hydroxyacyl-CoA dehydrogenase is required for proper orientation of the catalytic histidine and structural integrity of the enzyme The Journal of biological chemistry, 276 39, 36718Ibdah,J.A.; Paul,H.; Zhao,Y.; Binford,S.; Salleng,K.; Cline,M.; Matern,D.; Bennett,M.J.; Rinaldo,P.; Strauss,A.W. 2001. Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death The Journal of clinical investigation, 107 11, 1403Ibdah,J.A.; Zhao,Y.; Viola,J.; Gibson,B.; Bennett,M.J.; Strauss,A.W. 2001. Molecular prenatal diagnosis in families with fetal mitochondrial trifunctional protein mutations The Journal of pediatrics, 138 3, 396Jones,P.M.; Moffitt,M.; Joseph,D.; Harthcock,P.A.; Boriack,R.L.; Ibdah,J.A.; Strauss,A.W.; Bennett,M.J. 2001. Accumulation of free 3-hydroxy fatty acids in the culture media of fibroblasts from patients deficient in long-chain l-3-hydroxyacyl-CoA dehydrogenase: a useful diagnostic aid Clinical chemistry, 47 7, 1190Matern,D.; Schehata,B.M.; Shekhawa,P.; Strauss,A.W.; Bennett,M.J.; Rinaldo,P. 2001. Placental floor infarction complicating the pregnancy of a fetus with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency Molecular genetics and metabolism, 72 3, 265Wood,J.C.; Magera,M.J.; Rinaldo,P.; Seashore,M.R.; Strauss,A.W.; Friedman,A. 2001. Diagnosis of very long chain acyl-dehydrogenase deficiency from an infant's newborn screening card Pediatrics, 108 1, E19Woodside,K.J.; Knisely,A.S.; Strauss,A.W.; Gugliuzza,K.K.; Daller,J.A. 2001. Progression of hepatic damage during cold storage after procurement in a liver and kidney donor with HELLP syndrome Transplantation, 72 12, 1990Yoon,H.R.; Strauss,A.W.; Yoo,H.W. 2001. Sudden death in a Korean infant with very long-chain acyl-CoA dehydrogenase deficiency Journal of inherited metabolic disease, 24 3, 407Zytkovicz,T.H.; Fitzgerald,E.F.; Marsden,D.; Larson,C.A.; Shih,V.E.; Johnson,D.M.; Strauss,A.W.; Comeau,A.M.; Eaton,R.B.; Grady,G.F. 2001. Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program Clinical chemistry, 47 11, 1945Barycki,J.J.; O'Brien,L.K.; Strauss,A.W.; Banaszak,L.J. 2000. Sequestration of the active site by interdomain shifting. Crystallographic and spectroscopic evidence for distinct conformations of L-3-hydroxyacyl-CoA dehydrogenase The Journal of biological chemistry, 275 35, 27186Bennett,M.J.; Rinaldo,P.; Strauss,A.W. 2000. Inborn errors of mitochondrial fatty acid oxidation Critical reviews in clinical laboratory sciences, 37 1, 1Schlattner,U.; Eder,M.; Dolder,M.; Khuchua,Z.A.; Strauss,A.W.; Wallimann,T. 2000. Divergent enzyme kinetics and structural properties of the two human mitochondrial creatine kinase isoenzymes Biological chemistry, 381 11, 1063Barycki,J.J.; O'Brien,L.K.; Birktoft,J.J.; Strauss,A.W.; Banaszak,L.J. 1999. Pig heart short chain L-3-hydroxyacyl-CoA dehydrogenase revisited: sequence analysis and crystal structure determination Protein science : a publication of the Protein Society, 8 10, 2010Barycki,J.J.; O'Brien,L.K.; Bratt,J.M.; Zhang,R.; Sanishvili,R.; Strauss,A.W.; Banaszak,L.J. 1999. Biochemical characterization and crystal structure determination of human heart short chain L-3-hydroxyacyl-CoA dehydrogenase provide insights into catalytic mechanism Biochemistry, 38 18, 5786Hahn,S.H.; Lee,E.H.; Jung,J.W.; Hong,C.H.; Yoon,H.R.; Rinaldo,P.; Sims,H.; Gibson,B.; Strauss,A.W. 1999. Very long chain acyl coenzyme A dehydrogenase deficiency in a 5-month-old Korean boy: identification of a novel mutation The Journal of pediatrics, 135 2 Pt 1, 250Ibdah,J.A.; Bennett,M.J.; Rinaldo,P.; Zhao,Y.; Gibson,B.; Sims,H.F.; Strauss,A.W. 1999. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women The New England journal of medicine, 340 22, 1723Ibdah,J.A.; Dasouki,M.J.; Strauss,A.W. 1999. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: variable expressivity of maternal illness during pregnancy and unusual presentation with infantile cholestasis and hypocalcaemia Journal of inherited metabolic disease, 22 7, 811Matern,D.; Strauss,A.W.; Hillman,S.L.; Mayatepek,E.; Millington,D.S.; Trefz,F.K. 1999. Diagnosis of mitochondrial trifunctional protein deficiency in a blood spot from the newborn screening card by tandem mass spectrometry and DNA analysis Pediatric research, 46 1, 45Mathur,A.; Sims,H.F.; Gopalakrishnan,D.; Gibson,B.; Rinaldo,P.; Vockley,J.; Hug,G.; Strauss,A.W. 1999. Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death Circulation, 99 10, 1337Qin,W.; Khuchua,Z.; Boero,J.; Payne,R.M.; Strauss,A.W. 1999. Oxidative myocytes of heart and skeletal muscle express abundant sarcomeric mitochondrial creatine kinase The Histochemical journal, 31 6, 357Strauss,A.W.; Bennett,M.J.; Rinaldo,P.; Sims,H.F.; O'Brien,L.K.; Zhao,Y.; Gibson,B.; Ibdah,J. 1999. Inherited long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and a fetal-maternal interaction cause maternal liver disease and other pregnancy complications Seminars in perinatology, 23 2, 100Zhou,Y.; Kelly,D.P.; Strauss,A.W.; Sims,H.; Zhang,Z. 1999. Characterization of the human very-long-chain acyl-CoA dehydrogenase gene promoter region: a role for activator protein 2 Molecular genetics and metabolism, 68 4, 481Benson,D.W.; Sharkey,A.; Fatkin,D.; Lang,P.; Basson,C.T.; McDonough,B.; Strauss,A.W.; Seidman,J.G.; Seidman,C.E. 1998. Reduced penetrance, variable expressivity, and genetic heterogeneity of familial atrial septal defects Circulation, 97 20, 2043Ibdah,J.A.; Tein,I.; Dionisi-Vici,C.; Bennett,M.J.; IJlst,L.; Gibson,B.; Wanders,R.J.; Strauss,A.W. 1998. Mild trifunctional protein deficiency is associated with progressive neuropathy and myopathy and suggests a novel genotype-phenotype correlation The Journal of clinical investigation, 102 6, 1193Khuchua,Z.A.; Qin,W.; Boero,J.; Cheng,J.; Payne,R.M.; Saks,V.A.; Strauss,A.W. 1998. Octamer formation and coupling of cardiac sarcomeric mitochondrial creatine kinase are mediated by charged N-terminal residues The Journal of biological chemistry, 273 36, 22990Qin,W.; Khuchua,Z.; Cheng,J.; Boero,J.; Payne,R.M.; Strauss,A.W. 1998. Molecular characterization of the creatine kinases and some historical perspectives Molecular and cellular biochemistry, 184 1-2, 153Strauss, Arnold W. 1998. The molecular basis of congenital cardiac disease. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual, 1 , 179-188Strauss,A.W. 1998. The molecular basis of congenital cardiac disease , 1 , 179Eder,M.; Krautle,F.; Dong,Y.; Vock,P.; Kieweg,V.; Kim,J.J.; Strauss,A.W.; Ghisla,S. 1997. Characterization of human and pig kidney long-chain-acyl-CoA dehydrogenases and their role in beta-oxidation European journal of biochemistry / FEBS, 245 3, 600Qin,W.; Khuchua,Z.; Klein,S.C.; Strauss,A.W. 1997. Elements regulating cardiomyocyte expression of the human sarcomeric mitochondrial creatine kinase gene in transgenic mice The Journal of biological chemistry, 272 40, 25210Zhang,Z.; Zhou,Y.; Mendelsohn,N.J.; Bauer,G.S.; Strauss,A.W. 1997. Regulation of the human long chain acyl-CoA dehydrogenase gene by nuclear hormone receptor transcription factors Biochimica et biophysica acta, 1350 1, 53Bromberg,B.I.; Mazziotti,M.V.; Canter,C.E.; Spray,T.L.; Strauss,A.W.; Foglia,R.P. 1996. Recognition and management of nonpenetrating cardiac trauma in children The Journal of pediatrics, 128 4, 536Isaacs,J.D.,Jr; Sims,H.F.; Powell,C.K.; Bennett,M.J.; Hale,D.E.; Treem,W.R.; Strauss,A.W. 1996. Maternal acute fatty liver of pregnancy associated with fetal trifunctional protein deficiency: molecular characterization of a novel maternal mutant allele Pediatric research, 40 3, 393Johnson,M.C.; Watson,M.S.; Strauss,A.W. 1996. Chromosome 22q11 monosomy and the genetic basis of congenital heart disease The Journal of pediatrics, 129 1, 1Strauss,A.W.; Johnson,M.C. 1996. The genetic basis of pediatric cardiovascular disease Seminars in perinatology, 20 6, 564Brackett,J.C.; Sims,H.F.; Rinaldo,P.; Shapiro,S.; Powell,C.K.; Bennett,M.J.; Strauss,A.W. 1995. Two alpha subunit donor splice site mutations cause human trifunctional protein deficiency The Journal of clinical investigation, 95 5, 2076Johnson,M.C.; Payne,R.M.; Grant,J.W.; Strauss,A.W. 1995. The genetic basis of paediatric heart disease Annals of Medicine, 27 3, 289Johnson,M.C.; Strauss,A.W.; Dowton,S.B.; Spray,T.L.; Huddleston,C.B.; Wood,M.K.; Slaugh,R.A.; Watson,M.S. 1995. Deletion within chromosome 22 is common in patients with absent pulmonary valve syndrome The American Journal of Cardiology, 76 1, 66Johnson,M.C.; Watson,M.S.; Strauss,A.W.; Spray,T.L. 1995. Anomalous origin of the right pulmonary artery from the aorta and CATCH 22 syndrome The Annals of Thoracic Surgery, 60 3, 681Leone,T.C.; Cresci,S.; Carter,M.E.; Zhang,Z.; Lala,D.S.; Strauss,A.W.; Kelly,D.P. 1995. The human medium chain Acyl-CoA dehydrogenase gene promoter consists of a complex arrangement of nuclear receptor response elements and Sp1 binding sites The Journal of biological chemistry, 270 27, 16308Leone,T.C.; Cresci,S.; Carter,M.E.; Zhang,Z.; Lala,D.S.; Strauss,A.W.; Kelly,D.P. 1995. The human medium chain acyl-CoA dehydrogenase gene promoter consists of a complex arrangement of nuclear receptor response elements and Sp1 binding sites The Journal of biological chemistry, 270 41, 24622Payne,R.M.; Johnson,M.C.; Grant,J.W.; Strauss,A.W. 1995. Toward a molecular understanding of congenital heart disease Circulation, 91 2, 494Peterson,K.L.; Sergienko,E.E.; Wu,Y.; Kumar,N.R.; Strauss,A.W.; Oleson,A.E.; Muhonen,W.W.; Shabb,J.B.; Srivastava,D.K. 1995. Recombinant human liver medium-chain acyl-CoA dehydrogenase: purification, characterization, and the mechanism of interactions with functionally diverse C8-CoA molecules Biochemistry, 34 45, 14942Sims,H.F.; Brackett,J.C.; Powell,C.K.; Treem,W.R.; Hale,D.E.; Bennett,M.J.; Gibson,B.; Shapiro,S.; Strauss,A.W. 1995. The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy Proceedings of the National Academy of Sciences of the United States of America, 92 3, 841Strauss,A.W.; Powell,C.K.; Hale,D.E.; Anderson,M.M.; Ahuja,A.; Brackett,J.C.; Sims,H.F. 1995. Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood Proceedings of the National Academy of Sciences of the United States of America, 92 23, 10496Weinberger,M.J.; Rinaldo,P.; Strauss,A.W.; Bennett,M.J. 1995. Intact alpha-subunit is required for membrane-binding of human mitochondrial trifunctional beta-oxidation protein, but is not necessary for conferring 3-ketoacyl-CoA thiolase activity to the beta-subunit Biochemical and biophysical research communications, 209 1, 47Ziadeh,R.; Hoffman,E.P.; Finegold,D.N.; Hoop,R.C.; Brackett,J.C.; Strauss,A.W.; Naylor,E.W. 1995. Medium chain acyl-CoA dehydrogenase deficiency in Pennsylvania: neonatal screening shows high incidence and unexpected mutation frequencies Pediatric research, 37 5, 675Brackett,J.C.; Sims,H.F.; Steiner,R.D.; Nunge,M.; Zimmerman,E.M.; deMartinville,B.; Rinaldo,P.; Slaugh,R.; Strauss,A.W. 1994. A novel mutation in medium chain acyl-CoA dehydrogenase causes sudden neonatal death The Journal of clinical investigation, 94 4, 1477Djordjevic,S.; Dong,Y.; Paschke,R.; Frerman,F.E.; Strauss,A.W.; Kim,J.J. 1994. Identification of the catalytic base in long chain acyl-CoA dehydrogenase Biochemistry, 33 14, 4258Johnson,M.C.; Strauss,A.W. 1994. The jury is still out regarding balloon therapy for native aortic coarctation Journal of the American College of Cardiology, 24 6, 1589Kelly,D.P.; Strauss,A.W. 1994. Inherited cardiomyopathies The New England journal of medicine, 330 13, 913O'Shea,D.L.; Gast,M.J.; Murdock,G.L.; Payne,R.M.; Strauss,A.W. 1994. Expression of engineered human 17 beta-estradiol dehydrogenase in a prokaryotic system Journal of the Society for Gynecologic Investigation, 1 2, 143Payne,R.M.; Strauss,A.W. 1994. Developmental expression of sarcomeric and ubiquitous mitochondrial creatine kinase is tissue-specific Biochimica et biophysica acta, 1219 1, 33Payne,R.M.; Strauss,A.W. 1994. Expression of the mitochondrial creatine kinase genes Molecular and cellular biochemistry, 133-134 , 235Andresen,B.S.; Kolvraa,S.; Bross,P.; Bolund,L.; Curtis,D.; Eiberg,H.; Zhang,Z.; Kelly,D.P.; Strauss,A.W.; Gregersen,N. 1993. A silent A to G mutation in exon 11 of the medium-chain acyl-CoA dehydrogenase (MCAD) gene Human molecular genetics, 2 4, 488Hainline,B.E.; Kahlenbeck,D.J.; Grant,J.; Strauss,A.W. 1993. Tissue specific and developmental expression of rat long-and medium-chain acyl-CoA dehydrogenases Biochimica et biophysica acta, 1216 3, 460Johnson,M.C.; Canter,C.E.; Strauss,A.W.; Spray,T.L. 1993. Repair of coarctation of the aorta in infancy: comparison of surgical and balloon angioplasty American Heart Journal, 125 2 Pt 1, 464Payne,R.M.; Friedman,D.L.; Grant,J.W.; Perryman,M.B.; Strauss,A.W. 1993. Creatine kinase isoenzymes are highly regulated during pregnancy in rat uterus and placenta The American Journal of Physiology, 265 4 Pt 1, E624Whelan,A.J.; Strauss,A.W.; Hale,D.E.; Mendelsohn,N.J.; Kelly,D.P. 1993. Expression and characterization of human mutant (glutamic acid304) medium-chain acyl-coenzyme A dehydrogenase in mammalian cells Pediatric research, 34 5, 694Zhang,Z.; Kolvraa,S.; Zhou,Y.; Kelly,D.P.; Gregersen,N.; Strauss,A.W. 1993. Three RFLPs defining a haplotype associated with the common mutation in human medium-chain acyl-CoA dehydrogenase (MCAD) deficiency occur in Alu repeats American Journal of Human Genetics, 52 6, 1111Ghisla,S.; Engst,S.; Moll,M.; Bross,P.; Strauss,A.W.; Kim,J.J. 1992. Alpha, beta-dehydrogenation by acyl-CoA dehydrogenases: role of functional groups at the active center Progress in clinical and biological research, 375 , 127Kelly,D.P.; Hale,D.E.; Rutledge,S.L.; Ogden,M.L.; Whelan,A.J.; Zhang,Z.; Strauss,A.W. 1992. Molecular basis of inherited medium-chain acyl-CoA dehydrogenase deficiency causing sudden child death Journal of inherited metabolic disease, 15 2, 171Kelly,D.P.; Whelan,A.J.; Hale,D.E.; Rinaldo,P.; Rutledge,S.L.; Zhang,Z.; Strauss,A.W. 1992. Molecular characterization of medium-chain acyl-CoA dehydrogenase deficiency causing sudden death Progress in clinical and biological research, 375 , 463Kelly,D.P.; Zhang,Z.; Raisher,B.D.; Ogden,M.L.; Strauss,A.W. 1992. Identification and characterization of the 5' regulatory region of the human medium-chain acyl-CoA dehydrogenase (MCAD) gene Progress in clinical and biological research, 375 , 143Raisher,B.D.; Grant,J.W.; Martin,T.C.; Strauss,A.W.; Spray,T.L. 1992. Complete repair of total anomalous pulmonary venous connection in infancy The Journal of thoracic and cardiovascular surgery, 104 2, 443Raisher,B.D.; Gulick,T.; Zhang,Z.; Strauss,A.W.; Moore,D.D.; Kelly,D.P. 1992. Identification of a novel retinoid-responsive element in the promoter region of the medium chain acyl-coenzyme A dehydrogenase gene The Journal of biological chemistry, 267 28, 20264Su,C.Y.; Payne,M.; Strauss,A.W.; Dillmann,W.H. 1992. Selective reduction of creatine kinase subunit mRNAs in striated muscle of diabetic rats The American Journal of Physiology, 263 2 Pt 1, E310Tanaka,K.; Yokota,I.; Coates,P.M.; Strauss,A.W.; Kelly,D.P.; Zhang,Z.; Gregersen,N.; Andresen,B.S.; Matsubara,Y.; Curtis,D. 1992. Mutations in the medium chain acyl-CoA dehydrogenase (MCAD) gene Human mutation, 1 4, 271Zhang,Z.F.; Kelly,D.P.; Kim,J.J.; Zhou,Y.Q.; Ogden,M.L.; Whelan,A.J.; Strauss,A.W. 1992. Structural organization and regulatory regions of the human medium-chain acyl-CoA dehydrogenase gene Biochemistry, 31 1, 81Fontanet,H.L.; Trask,R.V.; Haas,R.C.; Strauss,A.W.; Abendschein,D.R.; Billadello,J.J. 1991. Regulation of expression of M, B, and mitochondrial creatine kinase mRNAs in the left ventricle after pressure overload in rats Circulation research, 68 4, 1007Gregersen,N.; Andresen,B.S.; Bross,P.; Winter,V.; Rudiger,N.; Engst,S.; Christensen,E.; Kelly,D.; Strauss,A.W.; Kolvraa,S. 1991. Molecular characterization of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency: identification of a lys329 to glu mutation in the MCAD gene, and expression of inactive mutant enzyme protein in E. coli Human genetics, 86 6, 545Gregersen,N.; Andresen,B.S.; Bross,P.; Winter,V.; Rudiger,N.; Engst,S.; Ghisla,S.; Christensen,E.; Kelly,D.; Strauss,A.W. 1991. Characterization of a disease-causing Lys329 to Glu mutation in 16 patients with medium-chain acyl-CoA dehydrogenase deficiency Journal of inherited metabolic disease, 14 3, 314Klein,S.C.; Haas,R.C.; Perryman,M.B.; Billadello,J.J.; Strauss,A.W. 1991. Regulatory element analysis and structural characterization of the human sarcomeric mitochondrial creatine kinase gene The Journal of biological chemistry, 266 27, 18058Murphy,A.M.; Jones,L.,2nd; Sims,H.F.; Strauss,A.W. 1991. Molecular cloning of rat cardiac troponin I and analysis of troponin I isoform expression in developing rat heart Biochemistry, 30 3, 707Payne,R.M.; Haas,R.C.; Strauss,A.W. 1991. Structural characterization and tissue-specific expression of the mRNAs encoding isoenzymes from two rat mitochondrial creatine kinase genes Biochimica et biophysica acta, 1089 3, 352Shackelford,P.G.; Strauss,A.W. 1991. Kawasaki syndrome The New England journal of medicine, 324 23, 1664Agrawal,H.C.; Agrawal,D.; Strauss,A.W. 1990. Cleavage of the P0 glycoprotein of the rat peripheral nerve myelin: tentative identification of cleavage site and evidence for the precursor-product relationship Neurochemical research, 15 10, 993Bross,P.; Engst,S.; Strauss,A.W.; Kelly,D.P.; Rasched,I.; Ghisla,S. 1990. Characterization of wild-type and an active site mutant of human medium chain acyl-CoA dehydrogenase after expression in Escherichia coli The Journal of biological chemistry, 265 13, 7116Haas,R.C.; Strauss,A.W. 1990. Separate nuclear genes encode sarcomere-specific and ubiquitous human mitochondrial creatine kinase isoenzymes The Journal of biological chemistry, 265 12, 6921Kelly,D.P.; Strauss,A.W. 1990. The tissue-specific and developmental regulation of expression of rat medium-chain acyl-CoA dehydrogenase mRNA Progress in clinical and biological research, 321 , 599Kelly,D.P.; Whelan,A.J.; Ogden,M.L.; Alpers,R.; Zhang,Z.F.; Bellus,G.; Gregersen,N.; Dorland,L.; Strauss,A.W. 1990. Molecular characterization of inherited medium-chain acyl-CoA dehydrogenase deficiency Proceedings of the National Academy of Sciences of the United States of America, 87 23, 9236Long,C.A.; Bauer,G.S.; Lowe,M.E.; Strauss,A.W.; Gast,M.J. 1990. Isolation and characterization of the gene from a human genome encoding 17 beta-estradiol dehydrogenase: a comparison of Jar and BeWo choriocarcinoma cell lines American Journal of Obstetrics and Gynecology, 163 6 Pt 1, 1976Lowe,M.; Strauss,A.W.; Alpers,R.; Seetharam,S.; Alpers,D.H. 1990. Molecular cloning and expression of a cDNA encoding the membrane-associated rat intestinal alkaline phosphatase Biochimica et biophysica acta, 1037 2, 170Lowe,M.E.; Rosenblum,J.L.; McEwen,P.; Strauss,A.W. 1990. Cloning and characterization of the human colipase cDNA Biochemistry, 29 3, 823Lowe,M.E.; Strauss,A.W. 1990. Expression of a Nagao-type, phosphatidylinositol-glycan anchored alkaline phosphatase in human choriocarcinomas Cancer research, 50 13, 3956Strauss,A.W.; Duran,M.; Zhang,Z.F.; Alpers,R.; Kelly,D.P. 1990. Molecular analysis of medium chain acyl-CoA dehydrogenase deficiency Progress in clinical and biological research, 321 , 609Strauss,A.W.; Goldring,D. 1990. Coarctation of the aorta in infants Circulation, 82 5, 1882Billadello,J.J.; Fontanet,H.L.; Strauss,A.W.; Abendschein,D.R. 1989. Characterization of MB creatine kinase isoform conversion in vitro and in vivo in dogs The Journal of clinical investigation, 83 5, 1637Chu,T.W.; Eftime,R.; Sztul,E.; Strauss,A.W. 1989. Synthetic transit peptides inhibit import and processing of mitochondrial precursor proteins The Journal of biological chemistry, 264 16, 9552Gast,M.J.; Sims,H.F.; Murdock,G.L.; Gast,P.M.; Strauss,A.W. 1989. Isolation and sequencing of a complementary deoxyribonucleic acid clone encoding human placental 17 beta-estradiol dehydrogenase: identification of the putative cofactor binding site American Journal of Obstetrics and Gynecology, 161 6 Pt 1, 1726Haas,R.C.; Korenfeld,C.; Zhang,Z.F.; Perryman,B.; Roman,D.; Strauss,A.W. 1989. Isolation and characterization of the gene and cDNA encoding human mitochondrial creatine kinase The Journal of biological chemistry, 264 5, 2890Kelly,D.P.; Gordon,J.I.; Alpers,R.; Strauss,A.W. 1989. The tissue-specific expression and developmental regulation of two nuclear genes encoding rat mitochondrial proteins. Medium chain acyl-CoA dehydrogenase and mitochondrial malate dehydrogenase The Journal of biological chemistry, 264 32, 18921Lowe,M.E.; Rosenblum,J.L.; Strauss,A.W. 1989. Cloning and characterization of human pancreatic lipase cDNA The Journal of biological chemistry, 264 33, 20042Sztul,E.S.; Chu,T.W.; Strauss,A.W.; Rosenberg,L.E. 1989. Translocation of precursor proteins into the mitochondrial matrix occurs through an environment accessible to aqueous perturbants Journal of cell science, 94 ( Pt 4) Pt 4, 695Griffin,M.L.; Hernandez,A.; Martin,T.C.; Goldring,D.; Bolman,R.M.; Spray,T.L.; Strauss,A.W. 1988. Dilated cardiomyopathy in infants and children Journal of the American College of Cardiology, 11 1, 139Stallings,R.L.; Olson,E.; Strauss,A.W.; Thompson,L.H.; Bachinski,L.L.; Siciliano,M.J. 1988. Human creatine kinase genes on chromosomes 15 and 19, and proximity of the gene for the muscle form to the genes for apolipoprotein C2 and excision repair American Journal of Human Genetics, 43 2, 144Sztul,E.S.; Chu,T.W.; Strauss,A.W.; Rosenberg,L.E. 1988. Import of the malate dehydrogenase precursor by mitochondria. Cleavage within leader peptide by matrix protease leads to formation of intermediate-sized form The Journal of biological chemistry, 263 24, 12085Trask,R.V.; Strauss,A.W.; Billadello,J.J. 1988. Developmental regulation and tissue-specific expression of the human muscle creatine kinase gene The Journal of biological chemistry, 263 32, 17142Abendschein,D.R.; Serota,H.; Plummer,T.H.,Jr; Amiraian,K.; Strauss,A.W.; Sobel,B.E.; Jaffe,A.S. 1987. Conversion of MM creatine kinase isoforms in human plasma by carboxypeptidase N The Journal of laboratory and clinical medicine, 110 6, 798Chu,T.W.; Grant,P.M.; Strauss,A.W. 1987. Mutation of a neutral amino acid in the transit peptide of rat mitochondrial malate dehydrogenase abolishes binding and import The Journal of biological chemistry, 262 32, 15759Chu,T.W.; Grant,P.M.; Strauss,A.W. 1987. The role of arginine residues in the rat mitochondrial malate dehydrogenase transit peptide The Journal of biological chemistry, 262 26, 12806Grace,A.M.; Gualdoni,J.; Strauss,A.W.; Sobel,B.E. 1987. Quantification of isoforms of plasma MM creatine kinase (CK) with an immunoblot procedure Catheterization and cardiovascular diagnosis, 13 1, 26Grant,P.M.; Roderick,S.L.; Grant,G.A.; Banaszak,L.J.; Strauss,A.W. 1987. Comparison of the precursor and mature forms of rat heart mitochondrial malate dehydrogenase Biochemistry, 26 1, 128Hortin,G.; Fok,K.F.; Toren,P.C.; Strauss,A.W. 1987. Sulfation of a tyrosine residue in the plasmin-binding domain of alpha 2-antiplasmin The Journal of biological chemistry, 262 7, 3082Kelly,D.P.; Kim,J.J.; Billadello,J.J.; Hainline,B.E.; Chu,T.W.; Strauss,A.W. 1987. Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue Proceedings of the National Academy of Sciences of the United States of America, 84 12, 4068Seetharam,S.; Ovitt,C.; Strauss,A.W.; Rubin,D.; Alpers,D.H. 1987. Fat feeding stimulates only one of the two mRNAs encoding rat intestinal membranous and secreted alkaline phosphatase Biochemical and biophysical research communications, 145 1, 363Alpers,D.H.; Helms,D.; Seetharam,S.; May,V.L.; Strauss,A.W. 1986. In vitro translation of intestinal sucrase-isomaltase and glucoamylase Biochemical and biophysical research communications, 134 1, 37Billadello,J.J.; Kelly,D.P.; Roman,D.G.; Strauss,A.W. 1986. The complete nucleotide sequence of canine brain B creatine kinase mRNA: homology in the coding and 3' noncoding regions among species Biochemical and biophysical research communications, 138 1, 392Canter,C.E.; Martin,T.C.; Spray,T.L.; Weldon,C.S.; Strauss,A.W. 1986. Scimitar syndrome in childhood The American Journal of Cardiology, 58 7, 652Gordon,J.I.; Sims,H.F.; Strauss,A.W.; Scanu,A.M.; Edelstein,C.; Byrne,R.E. 1986. Proteolytic processing and compartmentalization of the primary translation products of mammalian apolipoprotein mRNAs CRC critical reviews in biochemistry, 20 1, 37Grant,P.M.; Tellam,J.; May,V.L.; Strauss,A.W. 1986. Isolation and nucleotide sequence of a cDNA clone encoding rat mitochondrial malate dehydrogenase Nucleic acids research, 14 15, 6053Hortin,G.; Chan,A.C.; Fok,K.F.; Strauss,A.W.; Atkinson,J.P. 1986. Sequence analysis of the COOH terminus of the alpha-chain of the fourth component of human complement. Identification of the site of its extracellular cleavage The Journal of biological chemistry, 261 19, 9065Hortin,G.; Folz,R.; Gordon,J.I.; Strauss,A.W. 1986. Characterization of sites of tyrosine sulfation in proteins and criteria for predicting their occurrence Biochemical and biophysical research communications, 141 1, 326Hortin,G.; Green,E.D.; Baenziger,J.U.; Strauss,A.W. 1986. Sulphation of proteins secreted by a human hepatoma-derived cell line. Sulphation of N-linked oligosaccharides on alpha 2HS-glycoprotein The Biochemical journal, 235 2, 407Hortin,G.; Sims,H.; Strauss,A.W. 1986. Identification of the site of sulfation of the fourth component of human complement The Journal of biological chemistry, 261 4, 1786Hortin,G.; Strauss,A.W. 1986. Effects of acidotropic compounds on the secretory pathway: inhibition of secretion and processing of the third and fourth components of complement Biochemical and biophysical research communications, 136 2, 603Hortin,G.; Tollefsen,D.M.; Strauss,A.W. 1986. Identification of two sites of sulfation of human heparin cofactor II The Journal of biological chemistry, 261 34, 15827Ovitt,C.E.; Strauss,A.W.; Alpers,D.H.; Chou,J.Y.; Boime,I. 1986. Expression of different-sized placental alkaline phosphatase mRNAs in placenta and choriocarcinoma cells Proceedings of the National Academy of Sciences of the United States of America, 83 11, 3781Billadello,J.J.; Roman,D.G.; Grace,A.M.; Sobel,B.E.; Strauss,A.W. 1985. The nature of post-translational formation of MM creatine kinase isoforms The Journal of biological chemistry, 260 28, 14988Gordon,J.I.; Sims,H.F.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1985. Extracellular processing of proapolipoprotein A-II in Hep G2 cell cultures is mediated by a 54-kDa protease immunologically related to cathepsin B The Journal of biological chemistry, 260 27, 14824Grace,A.M.; Strauss,A.W.; Sobel,B.E. 1985. Sensitive quantification of isoforms of canine MM creatine kinase with an immunoblot procedure suitable for large numbers of samples Analytical Biochemistry, 149 1, 209Hashimoto,H.; Abendschein,D.R.; Strauss,A.W.; Sobel,B.E. 1985. Early detection of myocardial infarction in conscious dogs by analysis of plasma MM creatine kinase isoforms Circulation, 71 2, 363Alpers,D.H.; Strauss,A.W.; Ockner,R.K.; Bass,N.M.; Gordon,J.I. 1984. Cloning of a cDNA encoding rat intestinal fatty acid binding protein Proceedings of the National Academy of Sciences of the United States of America, 81 2, 313Blaufuss,M.C.; Gordon,J.I.; Schonfeld,G.; Strauss,A.W.; Alpers,D.H. 1984. Biosynthesis of apolipoprotein C-III in rat liver and small intestinal mucosa The Journal of biological chemistry, 259 4, 2452Gordon,J.I.; Bisgaier,C.L.; Sims,H.F.; Sachdev,O.P.; Glickman,R.M.; Strauss,A.W. 1984. Biosynthesis of human preapolipoprotein A-IV The Journal of biological chemistry, 259 1, 468Gordon,J.I.; Sims,H.F.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1984. Human proapolipoprotein A-II is cleaved following secretion from Hep G2 cells by a thiol protease The Journal of biological chemistry, 259 24, 15556Hashimoto,H.; Grace,A.M.; Billadello,J.J.; Gross,R.W.; Strauss,A.W.; Sobel,B.E. 1984. Nondenaturing quantification of subforms of canine MM creatine kinase isoenzymes (isoforms) and their interconversion The Journal of laboratory and clinical medicine, 103 3, 470Leonard,W.J.; Strauss,A.W.; Go,M.F.; Alpers,D.H.; Gordon,J.I. 1984. Biosynthesis and compartmentalization of rat-intestinal vitamin-D-dependent calcium-binding protein European journal of biochemistry / FEBS, 139 3, 561Lowe,J.B.; Strauss,A.W.; Gordon,J.I. 1984. Expression of a mammalian fatty acid-binding protein in Escherichia coli The Journal of biological chemistry, 259 20, 12696Cockerham,J.T.; Martin,T.C.; Gutierrez,F.R.; Hartmann,A.F.,Jr; Goldring,D.; Strauss,A.W. 1983. Spontaneous closure of secundum atrial septal defect in infants and young children The American Journal of Cardiology, 52 10, 1267Edelstein,C.; Gordon,J.I.; Toscas,K.; Sims,H.F.; Strauss,A.W.; Scanu,A.M. 1983. In vitro conversion of proapoprotein A-I to apoprotein A-I. Partial characterization of an extracellular enzyme activity The Journal of biological chemistry, 258 19, 11430Gordon,J.I.; Alpers,D.H.; Ockner,R.K.; Strauss,A.W. 1983. The nucleotide sequence of rat liver fatty acid binding protein mRNA The Journal of biological chemistry, 258 5, 3356Gordon,J.I.; Budelier,K.A.; Sims,H.F.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1983. Biosynthesis of human preproapolipoprotein A-II The Journal of biological chemistry, 258 22, 14054Gordon,J.I.; Sims,H.F.; Lentz,S.R.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1983. Proteolytic processing of human preproapolipoprotein A-I. A proposed defect in the conversion of pro A-I to A-I in Tangier's disease The Journal of biological chemistry, 258 6, 4037Perryman,M.B.; Strauss,A.W.; Buettner,T.L.; Roberts,R. 1983. Molecular heterogeneity of creatine kinase isoenzymes Biochimica et biophysica acta, 747 3, 284Perryman,M.B.; Strauss,A.W.; Olson,J.; Roberts,R. 1983. In vitro translation of canine mitochondrial creatine kinase messenger RNA Biochemical and biophysical research communications, 110 3, 967Gordon,J.I.; Smith,D.P.; Alpers,D.H.; Strauss,A.W. 1982. Cloning of a complementary deoxyribonucleic acid encoding a portion of rat intestinal preapolipoprotein AIV messenger ribonucleic acid Biochemistry, 21 22, 5424Gordon,J.I.; Smith,D.P.; Alpers,D.H.; Strauss,A.W. 1982. Proteolytic processing of the primary translation product of rat intestinal apolipoprotein A-IV mRNA. Comparison with preproapolipoprotein A-I processing The Journal of biological chemistry, 257 14, 8418Gordon,J.I.; Smith,D.P.; Andy,R.; Alpers,D.H.; Schonfeld,G.; Strauss,A.W. 1982. The primary translation product of rat intestinal apolipoprotein A-I mRNA is an unusual preproprotein The Journal of biological chemistry, 257 2, 971Graves,C.B.; Munns,T.W.; Willingham,A.K.; Strauss,A.W. 1982. Rat factor X is synthesized as a single chain precursor inducible by prothrombin fragments The Journal of biological chemistry, 257 21, 13108Strauss,A.W.; Boime,I. 1982. Compartmentation of newly synthesized proteins CRC critical reviews in biochemistry, 12 3, 205Taysi,K.; Strauss,A.W.; Yang,V.; Padmalatha,C.; Marshall,R.E. 1982. Terminal deletion of the long arm of chromosome 10 : q26 to qter. Case report and review of literature Annales de Genetique, 25 3, 141Canter,C.E.; Bower,R.J.; Strauss,A.W. 1981. Atypical Kawasaki disease with aortic aneurysm Pediatrics, 68 6, 885Graves,C.B.; Munns,T.W.; Carlisle,T.L.; Grant,G.A.; Strauss,A.W. 1981. Induction of prothrombin synthesis by prothrombin fragments Proceedings of the National Academy of Sciences of the United States of America, 78 8, 4772Henry,C.G.; Goldring,D.; Hartmann,A.F.; Weldon,C.S.; Strauss,A.W. 1981. Treatment of d-transposition of the great arteries: management of hypoxemia after balloon atrial septostomy The American Journal of Cardiology, 47 2, 299Henry,C.G.; Gutierrez,F.; Lee,J.T.; Hartmann,A.F.; Bell,M.J.; Bower,R.J.; Strauss,A.W. 1981. Aortic thrombosis presenting as congestive heart failure: an umbilical artery catheter complication The Journal of pediatrics, 98 5, 820Henry,C.G.; Strauss,A.W.; Keating,J.P.; Hillman,R.E. 1981. Congestive cardiomyopathy associated with beta-ketothiolase deficiency The Journal of pediatrics, 99 5, 754Mumford,R.A.; Pickett,C.B.; Zimmerman,M.; Strauss,A.W. 1981. Protease activities present in wheat germ and rabbit reticulocyte lysates Biochemical and biophysical research communications, 103 2, 565Mumford,R.A.; Pierzchala,P.A.; Strauss,A.W.; Zimmerman,M. 1981. Purification of a membrane-bound metalloendopeptidase from porcine kidney that degrades peptide hormones Proceedings of the National Academy of Sciences of the United States of America, 78 11, 6623Mumford,R.A.; Strauss,A.W.; Powers,J.C.; Pierzchala,P.A.; Nishino,N.; Zimmerman,M. 1980. A zinc metalloendopeptidase associated with dog pancreatic membranes The Journal of biological chemistry, 255 6, 2227Strauss,A.W.; Zimmerman,M.; Mumford,R.A.; Alberts,A.W. 1980. Processing of pre-proalbumin and pre-placental lactogen Annals of the New York Academy of Sciences, 343 , 168Zimmerman,M.; Ashe,B.M.; Alberts,A.W.; Pierzchala,P.A.; Powers,J.C.; Nishino,N.; Strauss,A.W.; Mumford,R.A. 1980. Protease activities present in dog pancreatic membranes that process human pre-placental lactogen Annals of the New York Academy of Sciences, 343 , 405Haymond,M.W.; Strauss,A.W.; Arnold,K.J.; Bier,D.M. 1979. Glucose homeostasis in children with severe cyanotic congenital heart disease The Journal of pediatrics, 95 2, 220Jones,L.A.; Jordan,D.K.; Taysi,K.; Strauss,A.W.; Toth,J.K. 1979. Partial duplication of the long arm of chromosome 5: a case due to balanced paternal translocation and review of the literature Human genetics, 51 1, 37Jones,L.A.; Taysi,K.; Strauss,A.W.; Hartmann,A.F. 1979. Partial trisomy 13 as a result of de novo (6p;13q) translocation Human genetics, 48 2, 245Strauss,A.W.; Zimmerman,M.; Boime,I.; Ashe,B.; Mumford,R.A.; Alberts,A.W. 1979. Characterization of an endopeptidase involved in pre-protein processing Proceedings of the National Academy of Sciences of the United States of America, 76 9, 4225Taysi,K.; Noetzel,M.J.; Strauss,A.W. 1979. Presumptive long arm deletion of chromosome 8: a new syndrome? Human genetics, 51 1, 49Hernandez,A.; Strauss,A.W.; McKnight,R.; Hartmann,A.F.,Jr. 1978. Diagnosis of pulmonary arteriovenous fistula by contrast echocardiography The Journal of pediatrics, 93 2, 258Strauss,A.W.; Bennett,C.A.; Donohue,A.M.; Rodkey,J.A.; Boime,I.; Alberts,A.W. 1978. Conversion of rat pre-proalbumin to proalbumin in vitro by ascites membranes. Demonstration by NH2-TERMINAL SEQUENCE ANALYSIS The Journal of biological chemistry, 253 17, 6270Strauss,A.W.; Bennett,C.D.; Donohue,A.M.; Rodkey,J.A.; Alberts,A.W. 1977. Rat liver pre-proalbumin: complete amino acid sequence of the pre-piece. Analysis of the direct translation product of albumin messenger RNA The Journal of biological chemistry, 252 19, 6846Strauss,A.W.; Donohue,A.M.; Bennett,C.D.; Rodkey,J.A.; Alberts,A.W. 1977. Rat liver preproalbumin: in vitro synthesis and partial amino acid sequence Proceedings of the National Academy of Sciences of the United States of America, 74 4, 1358Alberts,A.W.; Strauss,A.W.; Hennessy,S.; Vagelos,P.R. 1975. Regulation of synthesis of hepatic fatty acid synthetase: binding of fatty acid synthetase antibodies to polysomes Proceedings of the National Academy of Sciences of the United States of America, 72 10, 3956Alderson,P.O.; Jost,R.G.; Strauss,A.W.; Boonvisut,S.; Markham,J. 1975. Radionuclide angiocardiography. Improved diagnosis and quantitation of left-to-right shunts using area ratio techniques in children Circulation, 51 6, 1136Strauss,A.W.; Alberts,A.W.; Hennessy,S.; Vagelos,P.R. 1975. Regulation of synthesis of hepatic fatty acid synthetase: polysomal translation in a cell-free system Proceedings of the National Academy of Sciences of the United States of America, 72 11, 4366Strauss,A.W.; Santa-Maria,M.; Goldring,D. 1975. Constrictive pericarditis in children American Journal of Diseases of Children (1960), 129 7, 822Strauss,A.W.; Escobedo,M.; Goldring,D. 1974. Continuous monitoring of arterial oxygen tension in the newborn infant The Journal of pediatrics, 85 2, 254Strauss,A.W.; Goldring,D. 1974. Editorial: Valve replacement in acute rheumatic heart disease The Journal of pediatrics, 84 5, 786Strauss,A.W.; Goldring,D.; Kissane,J.; Hernandez,A.; Hartmann,A.F.; McKnight,C.R.; Weldon,C.S. 1974. Valve replacement in acute rheumatic heart disease The Journal of thoracic and cardiovascular surgery, 67 4, 659Keating,J.P.; Lell,M.E.; Strauss,A.W.; Zarkowsky,H.; Smith,G.E. 1973. Infantile methemoglobinemia caused by carrot juice The New England journal of medicine, 288 16, 824STRAUSS,A.W.; STRAUSS,A.W.,Jr. 1952. Pseudomucinous cystadenocarcinoma of the ovary American Journal of Obstetrics and Gynecology, 64 6, 1382Bennett, Michael J; Russell, Laurie K; Tokunaga, Chonan; Narayan, Srinivas B; Tan, Lu; Seegmiller, Adam; Boriack, Richard L; Strauss, Arnold W . Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase. Molecular genetics and metabolism, 89 1-2, 74-9
Acehan, Devrim; Vaz, Frederic; Houtkooper, Riekelt H; James, Jeanne; Moore, Vicky; Tokunaga, Chonan; Kulik, Willem; Wansapura, Janaka; Toth, Matthew J; Strauss, Arnold; Khuchua, Zaza 2011. Cardiac and skeletal muscle defects in a mouse model of human Barth syndrome. The Journal of biological chemistry, 286 2, 899-908Britigan, Bradley; Strauss, Arnold; Susman, Jeff 2010. Salami science or editorial imperialism? The Journal of pediatrics, 157 3, 518-9; author reply Li, Changhong; Chen, Pan; Palladino, Andrew; Narayan, Srinivas; Russell, Laurie K; Sayed, Samir; Xiong, Guoxiang; Chen, Jie; Stokes, David; Butt, Yasmeen M; Jones, Patricia M; Collins, Heather W; Cohen, Noam A; Cohen, Akiva S; Nissim, Itzhak; Smith, Thomas J; Strauss, Arnold W; Matschinsky, Franz M; Bennett, Michael J; Stanley, Charles A 2010. Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase. The Journal of biological chemistry, 285 41, 31806-18Arnold, Georgianne L; Van Hove, Johan; Freedenberg, Debra; Strauss, Arnold; Longo, Nicola; Burton, Barbara; Garganta, Cheryl; Ficicioglu, Can; Cederbaum, Stephen; Harding, Cary; Boles, Richard G; Matern, Dietrich; Chakraborty, Pranesh; Feigenbaum, Annette 2009. A Delphi clinical practice protocol for the management of very long chain acyl-CoA dehydrogenase deficiency. Molecular genetics and metabolism, 96 3, 85-90Chappell, Tresa; Creech, C Buddy; Parra, David; Strauss, Arnold; Scholl, Frank; Whitney, Gina 2008. Presentation of pulmonary artery intimal sarcoma in an infant with a history of neonatal valvular pulmonic stenosis. The Annals of thoracic surgery, 85 3, 1092-4Hsu, Ho-Wen; Zytkovicz, Thomas H; Comeau, Anne Marie; Strauss, Arnold W; Marsden, Deborah; Shih, Vivian E; Grady, George F; Eaton, Roger B 2008. Spectrum of medium-chain acyl-CoA dehydrogenase deficiency detected by newborn screening. Pediatrics, 121 5, e1108-14Werdich, Andreas A; Baudenbacher, Franz; Dzhura, Igor; Jeyakumar, Loice H; Kannankeril, Prince J; Fleischer, Sidney; LeGrone, Alison; Milatovic, Dejan; Aschner, Michael; Strauss, Arnold W; Anderson, Mark E; Exil, Vernat J 2007. Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice. American journal of physiology. Heart and circulatory physiology, 292 5, H2202-11Werdich,A.A.; Baudenbacher,F.; Dzhura,I.; Jeyakumar,L.H.; Kannankeril,P.J.; Fleischer,S.; Legrone,A.; Milatovic,D.; Aschner,M.; Strauss,A.W.; Anderson,M.; Exil,V.J. 2007. Polymorphic Ventricular Tachycardia and Abnormal Calcium Handling in Very-Long-Chain Acyl-CoA Dehydrogenase Null Mice American Journal of Physiology. Heart and Circulatory Physiology, , Bennett,M.J.; Russell,L.K.; Tokunaga,C.; Narayan,S.B.; Tan,L.; Seegmiller,A.; Boriack,R.L.; Strauss,A.W. 2006. Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase Molecular genetics and metabolism, 89 1-2, 74Djouadi,F.; Aubey,F.; Schlemmer,D.; Gobin,S.; Laforet,P.; Wanders,R.J.; Strauss,A.W.; Bonnefont,J.P.; Bastin,J. 2006. Potential of fibrates in the treatment of fatty acid oxidation disorders: revival of classical drugs? Journal of inherited metabolic disease, 29 2-3, 341Exil, Vernat J; Gardner, Carla D; Rottman, Jeffrey N; Sims, Harold; Bartelds, Beatrijs; Khuchua, Zaza; Sindhal, Rekha; Ni, Gemin; Strauss, Arnold W 2006. Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase. American journal of physiology. Heart and circulatory physiology, 290 3, H1289-97Exil,V.J.; Gardner,C.D.; Rottman,J.N.; Sims,H.; Bartelds,B.; Khuchua,Z.; Sindhal,R.; Ni,G.; Strauss,A.W. 2006. Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase American journal of physiology.Heart and circulatory physiology, 290 3, H1289Hoffman, Jodi D; Steiner, Robert D; Paradise, Lori; Harding, Carey O; Ding, Li; Strauss, Arnold W; Kaplan, Paige 2006. Rhabdomyolysis in the military: recognizing late-onset very long-chain acyl Co-A dehydrogenase deficiency. Military medicine, 171 7, 657-8Hoffman,J.D.; Steiner,R.D.; Paradise,L.; Harding,C.O.; Ding,L.; Strauss,A.W.; Kaplan,P. 2006. Rhabdomyolysis in the military: recognizing late-onset very long-chain acyl Co-A dehydrogenase deficiency Military medicine, 171 7, 657Khuchua, Zaza; Yue, Zou; Batts, Lorene; Strauss, Arnold W 2006. A zebrafish model of human Barth syndrome reveals the essential role of tafazzin in cardiac development and function. Circulation research, 99 2, 201-8Khuchua,Z.; Yue,Z.; Batts,L.; Strauss,A.W. 2006. A zebrafish model of human Barth syndrome reveals the essential role of tafazzin in cardiac development and function Circulation research, 99 2, 201Liebig, Michaela; Schymik, Ina; Mueller, Martina; Wendel, Udo; Mayatepek, Ertan; Ruiter, Jos; Strauss, Arnold W; Wanders, Ronald J A; Spiekerkoetter, Ute 2006. Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels. Pediatrics, 118 3, 1065-9Liebig,M.; Gyenes,M.; Brauers,G.; Ruiter,J.P.; Wendel,U.; Mayatepek,E.; Strauss,A.W.; Wanders,R.J.; Spiekerkoetter,U. 2006. Carnitine supplementation induces long-chain acylcarnitine production--studies in the VLCAD-deficient mouse Journal of inherited metabolic disease, 29 2-3, 343Liebig,M.; Schymik,I.; Mueller,M.; Wendel,U.; Mayatepek,E.; Ruiter,J.; Strauss,A.W.; Wanders,R.J.; Spiekerkoetter,U. 2006. Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels Pediatrics, 118 3, 1065Liske, Michael R; Greeley, Christopher S; Law, David J; Reich, Jonathan D; Morrow, William R; Baldwin, H Scott; Graham, Thomas P; Strauss, Arnold W; Kavanaugh-McHugh, Ann L; Walsh, William F 2006. Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease. Pediatrics, 118 4, e1250-6Liske,M.R.; Greeley,C.S.; Law,D.J.; Reich,J.D.; Morrow,W.R.; Baldwin,H.S.; Graham,T.P.; Strauss,A.W.; Kavanaugh-McHugh,A.L.; Walsh,W.F.; Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease. 2006. Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease Pediatrics, 118 4, e1250Schymik, Ina; Liebig, Michaela; Mueller, Martina; Wendel, Udo; Mayatepek, Ertan; Strauss, Arnold W; Wanders, Ronald J A; Spiekerkoetter, Ute 2006. Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry. The Journal of pediatrics, 149 1, 128-30Schymik,I.; Liebig,M.; Mueller,M.; Wendel,U.; Mayatepek,E.; Strauss,A.W.; Wanders,R.J.; Spiekerkoetter,U. 2006. Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry The Journal of pediatrics, 149 1, 128Spiekerkoetter,U.; Ruiter,J.; Tokunaga,C.; Wendel,U.; Mayatepek,E.; Wijburg,F.A.; Strauss,A.W.; Wanders,R.J. 2006. Evidence for impaired gluconeogenesis in very long-chain acyl-CoA dehydrogenase-deficient mice Hormone and metabolic research.Hormon- und Stoffwechselforschung.Hormones et metabolisme, 38 10, 625Djouadi,F.; Aubey,F.; Schlemmer,D.; Ruiter,J.P.; Wanders,R.J.; Strauss,A.W.; Bastin,J. 2005. Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders Human molecular genetics, 14 18, 2695Shekhawat, Prem S; Matern, Dietrich; Strauss, Arnold W 2005. Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and management. Pediatric research, 57 5 Pt 2, 78R-86RShekhawat,P.S.; Matern,D.; Strauss,A.W. 2005. Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and management Pediatric research, 57 5 Pt 2, 78RSpiekerkoetter, Ute; Tokunaga, Chonan; Wendel, Udo; Mayatepek, Ertan; Ijlst, Lodewijk; Vaz, Frederic M; van Vlies, Naomi; Overmars, Henk; Duran, Marinus; Wijburg, Frits A; Wanders, Ronald J; Strauss, Arnold W 2005. Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. Pediatric research, 57 6, 760-4Spiekerkoetter,U.; Tokunaga,C.; Wendel,U.; Mayatepek,E.; Ijlst,L.; Vaz,F.M.; van Vlies,N.; Overmars,H.; Duran,M.; Wijburg,F.A.; Wanders,R.J.; Strauss,A.W. 2005. Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice Pediatric research, 57 6, 760Strauss, Arnold W 2005. Surprising? Perhaps not. Long-chain fatty acid oxidation during human fetal development. Pediatric research, 57 6, 753-4Strauss,A.W. 2005. Surprising? Perhaps not. Long-chain fatty acid oxidation during human fetal development Pediatric research, 57 6, 753McKinney,J.T.; Longo,N.; Hahn,S.H.; Matern,D.; Rinaldo,P.; Strauss,A.W.; Dobrowolski,S.F. 2004. Rapid, comprehensive screening of the human medium chain acyl-CoA dehydrogenase gene Molecular genetics and metabolism, 82 2, 112Spiekerkoetter, Ute; Bennett, Michael J; Ben-Zeev, Bruria; Strauss, Arnold W; Tein, Ingrid 2004. Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein. Muscle & nerve, 29 1, 66-72Spiekerkoetter, Ute; Khuchua, Zaza; Yue, Zou; Bennett, Michael J; Strauss, Arnold W 2004. General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnover. Pediatric research, 55 2, 190-6Spiekerkoetter,U.; Bennett,M.J.; Ben-Zeev,B.; Strauss,A.W.; Tein,I. 2004. Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein Muscle & nerve, 29 1, 66Spiekerkoetter,U.; Khuchua,Z.; Yue,Z.; Bennett,M.J.; Strauss,A.W. 2004. General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnover Pediatric research, 55 2, 190Spiekerkoetter,U.; Tokunaga,C.; Wendel,U.; Mayatepek,E.; Exil,V.; Duran,M.; Wijburg,F.A.; Wanders,R.J.; Strauss,A.W. 2004. Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress European journal of clinical investigation, 34 3, 191Spierkerkoetter,U.; Khuchua,Z.; Yue,Z.; Strauss,A.W. 2004. The early-onset phenotype of mitochondrial trifunctional protein deficiency: a lethal disorder with multiple tissue involvement Journal of inherited metabolic disease, 27 2, 294Strauss, Arnold W 2004. Tandem mass spectrometry in discovery of disorders of the metabolome. The Journal of clinical investigation, 113 3, 354-6Strauss,A.W. 2004. Tandem mass spectrometry in discovery of disorders of the metabolome The Journal of clinical investigation, 113 3, 354Tsirka, Anna E; Trinkaus, Kathryn; Chen, Su-Chiung; Lipshultz, Steven E; Towbin, Jeffrey A; Colan, Steven D; Exil, Vernat; Strauss, Arnold W; Canter, Charles E 2004. Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation. Journal of the American College of Cardiology, 44 2, 391-7Tsirka,A.E.; Trinkaus,K.; Chen,S.C.; Lipshultz,S.E.; Towbin,J.A.; Colan,S.D.; Exil,V.; Strauss,A.W.; Canter,C.E. 2004. Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation Journal of the American College of Cardiology, 44 2, 391Tyni, Tiina; Paetau, Anders; Strauss, Arnold W; Middleton, Bruce; Kivelä, Tero 2004. Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Pediatric research, 56 5, 744-50Tyni,T.; Paetau,A.; Strauss,A.W.; Middleton,B.; Kivela,T. 2004. Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency Pediatric research, 56 5, 744Boero,J.; Qin,W.; Cheng,J.; Woolsey,T.A.; Strauss,A.W.; Khuchua,Z. 2003. Restricted neuronal expression of ubiquitous mitochondrial creatine kinase: changing patterns in development and with increased activity Molecular and cellular biochemistry, 244 1-2, 69Exil, Vernat J; Roberts, Richard L; Sims, Harold; McLaughlin, Jacquelin E; Malkin, Robert A; Gardner, Carla D; Ni, Gemin; Rottman, Jeffrey N; Strauss, Arnold W 2003. Very-long-chain acyl-coenzyme a dehydrogenase deficiency in mice. Circulation research, 93 5, 448-55Exil,V.J.; Roberts,R.L.; Sims,H.; McLaughlin,J.E.; Malkin,R.A.; Gardner,C.D.; Ni,G.; Rottman,J.N.; Strauss,A.W. 2003. Very-long-chain acyl-coenzyme a dehydrogenase deficiency in mice Circulation research, 93 5, 448Khuchua,Z.; Wozniak,D.F.; Bardgett,M.E.; Yue,Z.; McDonald,M.; Boero,J.; Hartman,R.E.; Sims,H.; Strauss,A.W. 2003. Deletion of the N-terminus of murine map2 by gene targeting disrupts hippocampal ca1 neuron architecture and alters contextual memory Neuroscience, 119 1, 101Lee,J.E.; Yoon,H.R.; Paik,K.H.; Hwang,S.J.; Shim,J.W.; Chang,Y.S.; Park,W.S.; Strauss,A.W.; Jin,D.K. 2003. A case of mitochondrial trifunctional protein deficiency diagnosed by acylcarnitine profile and DNA analysis in a dried blood spot of a 4-day-old boy Journal of inherited metabolic disease, 26 4, 403Shekhawat, Prem; Bennett, Michael J; Sadovsky, Yoel; Nelson, D Michael; Rakheja, Dinesh; Strauss, Arnold W 2003. Human placenta metabolizes fatty acids: implications for fetal fatty acid oxidation disorders and maternal liver diseases. American journal of physiology. Endocrinology and metabolism, 284 6, E1098-105Shekhawat,P.; Bennett,M.J.; Sadovsky,Y.; Nelson,D.M.; Rakheja,D.; Strauss,A.W. 2003. Human placenta metabolizes fatty acids: implications for fetal fatty acid oxidation disorders and maternal liver diseases American journal of physiology.Endocrinology and metabolism, 284 6, E1098Spiekerkoetter, Ute; Sun, Bin; Khuchua, Zaza; Bennett, Michael J; Strauss, Arnold W 2003. Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations. Human mutation, 21 6, 598-607Spiekerkoetter, Ute; Sun, Bin; Zytkovicz, Thomas; Wanders, Ronald; Strauss, Arnold W; Wendel, Udo 2003. MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency. The Journal of pediatrics, 143 3, 335-42Spiekerkoetter,U.; Sun,B.; Khuchua,Z.; Bennett,M.J.; Strauss,A.W. 2003. Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations Human mutation, 21 6, 598Spiekerkoetter,U.; Sun,B.; Zytkovicz,T.; Wanders,R.; Strauss,A.W.; Wendel,U. 2003. MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency The Journal of pediatrics, 143 3, 335Strauss, Arnold; Lock, James E 2003. Pediatric cardiomyopathy--a long way to go. The New England journal of medicine, 348 17, 1703-5Hintz, Susan R; Matern, Dietrich; Strauss, Arnold; Bennett, Michael J; Hoyme, H Eugene; Schelley, Susan; Kobori, Joyce; Colby, Christopher; Lehman, Norman L; Enns, Gregory M 2002. Early neonatal diagnosis of long-chain 3-hydroxyacyl coenzyme a dehydrogenase and mitochondrial trifunctional protein deficiencies. Molecular genetics and metabolism, 75 2, 120-7Spiekerkoetter, Ute; Eeds, Angela; Yue, Zou; Haines, Jonathan; Strauss, Arnold W; Summar, Marshall 2002. Uniparental disomy of chromosome 2 resulting in lethal trifunctional protein deficiency due to homozygous alpha-subunit mutations. Human mutation, 20 6, 447-51Spiekerkoetter,U.; Eeds,A.; Yue,Z.; Haines,J.; Strauss,A.W.; Summar,M. 2002. Uniparental disomy of chromosome 2 resulting in lethal trifunctional protein deficiency due to homozygous alpha-subunit mutations Human mutation, 20 6, 447Straussberg, Rachel; Strauss, Arnold W 2002. A novel mutation of late-onset very-long-chain acyl-CoA dehydrogenase deficiency. Pediatric neurology, 27 2, 136-7Straussberg,R.; Strauss,A.W. 2002. A novel mutation of late-onset very-long-chain acyl-CoA dehydrogenase deficiency Pediatric neurology, 27 2, 136Yang, Zi; Yamada, Jennifer; Zhao, Yiwen; Strauss, Arnold W; Ibdah, Jamal A 2002. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease. JAMA : the journal of the American Medical Association, 288 17, 2163-6Yang, Zi; Zhao, Yiwen; Bennett, Michael J; Strauss, Arnold W; Ibdah, Jamal A 2002. Fetal genotypes and pregnancy outcomes in 35 families with mitochondrial trifunctional protein mutations. American journal of obstetrics and gynecology, 187 3, 715-20Yang,Z.; Yamada,J.; Zhao,Y.; Strauss,A.W.; Ibdah,J.A. 2002. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease JAMA : the journal of the American Medical Association, 288 17, 2163Yang,Z.; Zhao,Y.; Bennett,M.J.; Strauss,A.W.; Ibdah,J.A. 2002. Fetal genotypes and pregnancy outcomes in 35 families with mitochondrial trifunctional protein mutations American Journal of Obstetrics and Gynecology, 187 3, 715Albers,S.; Levy,H.L.; Irons,M.; Strauss,A.W.; Marsden,D. 2001. Compound heterozygosity in four asymptomatic siblings with medium-chain acyl-CoA dehydrogenase deficiency Journal of inherited metabolic disease, 24 3, 417Barycki,J.J.; O'Brien,L.K.; Strauss,A.W.; Banaszak,L.J. 2001. Glutamate 170 of human l-3-hydroxyacyl-CoA dehydrogenase is required for proper orientation of the catalytic histidine and structural integrity of the enzyme The Journal of biological chemistry, 276 39, 36718Ibdah,J.A.; Paul,H.; Zhao,Y.; Binford,S.; Salleng,K.; Cline,M.; Matern,D.; Bennett,M.J.; Rinaldo,P.; Strauss,A.W. 2001. Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death The Journal of clinical investigation, 107 11, 1403Ibdah,J.A.; Zhao,Y.; Viola,J.; Gibson,B.; Bennett,M.J.; Strauss,A.W. 2001. Molecular prenatal diagnosis in families with fetal mitochondrial trifunctional protein mutations The Journal of pediatrics, 138 3, 396Jones,P.M.; Moffitt,M.; Joseph,D.; Harthcock,P.A.; Boriack,R.L.; Ibdah,J.A.; Strauss,A.W.; Bennett,M.J. 2001. Accumulation of free 3-hydroxy fatty acids in the culture media of fibroblasts from patients deficient in long-chain l-3-hydroxyacyl-CoA dehydrogenase: a useful diagnostic aid Clinical chemistry, 47 7, 1190Matern,D.; Schehata,B.M.; Shekhawa,P.; Strauss,A.W.; Bennett,M.J.; Rinaldo,P. 2001. Placental floor infarction complicating the pregnancy of a fetus with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency Molecular genetics and metabolism, 72 3, 265Wood,J.C.; Magera,M.J.; Rinaldo,P.; Seashore,M.R.; Strauss,A.W.; Friedman,A. 2001. Diagnosis of very long chain acyl-dehydrogenase deficiency from an infant's newborn screening card Pediatrics, 108 1, E19Woodside,K.J.; Knisely,A.S.; Strauss,A.W.; Gugliuzza,K.K.; Daller,J.A. 2001. Progression of hepatic damage during cold storage after procurement in a liver and kidney donor with HELLP syndrome Transplantation, 72 12, 1990Yoon,H.R.; Strauss,A.W.; Yoo,H.W. 2001. Sudden death in a Korean infant with very long-chain acyl-CoA dehydrogenase deficiency Journal of inherited metabolic disease, 24 3, 407Zytkovicz,T.H.; Fitzgerald,E.F.; Marsden,D.; Larson,C.A.; Shih,V.E.; Johnson,D.M.; Strauss,A.W.; Comeau,A.M.; Eaton,R.B.; Grady,G.F. 2001. Tandem mass spectrometric analysis for amino, organic, and fatty acid disorders in newborn dried blood spots: a two-year summary from the New England Newborn Screening Program Clinical chemistry, 47 11, 1945Barycki,J.J.; O'Brien,L.K.; Strauss,A.W.; Banaszak,L.J. 2000. Sequestration of the active site by interdomain shifting. Crystallographic and spectroscopic evidence for distinct conformations of L-3-hydroxyacyl-CoA dehydrogenase The Journal of biological chemistry, 275 35, 27186Bennett,M.J.; Rinaldo,P.; Strauss,A.W. 2000. Inborn errors of mitochondrial fatty acid oxidation Critical reviews in clinical laboratory sciences, 37 1, 1Schlattner,U.; Eder,M.; Dolder,M.; Khuchua,Z.A.; Strauss,A.W.; Wallimann,T. 2000. Divergent enzyme kinetics and structural properties of the two human mitochondrial creatine kinase isoenzymes Biological chemistry, 381 11, 1063Barycki,J.J.; O'Brien,L.K.; Birktoft,J.J.; Strauss,A.W.; Banaszak,L.J. 1999. Pig heart short chain L-3-hydroxyacyl-CoA dehydrogenase revisited: sequence analysis and crystal structure determination Protein science : a publication of the Protein Society, 8 10, 2010Barycki,J.J.; O'Brien,L.K.; Bratt,J.M.; Zhang,R.; Sanishvili,R.; Strauss,A.W.; Banaszak,L.J. 1999. Biochemical characterization and crystal structure determination of human heart short chain L-3-hydroxyacyl-CoA dehydrogenase provide insights into catalytic mechanism Biochemistry, 38 18, 5786Hahn,S.H.; Lee,E.H.; Jung,J.W.; Hong,C.H.; Yoon,H.R.; Rinaldo,P.; Sims,H.; Gibson,B.; Strauss,A.W. 1999. Very long chain acyl coenzyme A dehydrogenase deficiency in a 5-month-old Korean boy: identification of a novel mutation The Journal of pediatrics, 135 2 Pt 1, 250Ibdah,J.A.; Bennett,M.J.; Rinaldo,P.; Zhao,Y.; Gibson,B.; Sims,H.F.; Strauss,A.W. 1999. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women The New England journal of medicine, 340 22, 1723Ibdah,J.A.; Dasouki,M.J.; Strauss,A.W. 1999. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: variable expressivity of maternal illness during pregnancy and unusual presentation with infantile cholestasis and hypocalcaemia Journal of inherited metabolic disease, 22 7, 811Matern,D.; Strauss,A.W.; Hillman,S.L.; Mayatepek,E.; Millington,D.S.; Trefz,F.K. 1999. Diagnosis of mitochondrial trifunctional protein deficiency in a blood spot from the newborn screening card by tandem mass spectrometry and DNA analysis Pediatric research, 46 1, 45Mathur,A.; Sims,H.F.; Gopalakrishnan,D.; Gibson,B.; Rinaldo,P.; Vockley,J.; Hug,G.; Strauss,A.W. 1999. Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death Circulation, 99 10, 1337Qin,W.; Khuchua,Z.; Boero,J.; Payne,R.M.; Strauss,A.W. 1999. Oxidative myocytes of heart and skeletal muscle express abundant sarcomeric mitochondrial creatine kinase The Histochemical journal, 31 6, 357Strauss,A.W.; Bennett,M.J.; Rinaldo,P.; Sims,H.F.; O'Brien,L.K.; Zhao,Y.; Gibson,B.; Ibdah,J. 1999. Inherited long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and a fetal-maternal interaction cause maternal liver disease and other pregnancy complications Seminars in perinatology, 23 2, 100Zhou,Y.; Kelly,D.P.; Strauss,A.W.; Sims,H.; Zhang,Z. 1999. Characterization of the human very-long-chain acyl-CoA dehydrogenase gene promoter region: a role for activator protein 2 Molecular genetics and metabolism, 68 4, 481Benson,D.W.; Sharkey,A.; Fatkin,D.; Lang,P.; Basson,C.T.; McDonough,B.; Strauss,A.W.; Seidman,J.G.; Seidman,C.E. 1998. Reduced penetrance, variable expressivity, and genetic heterogeneity of familial atrial septal defects Circulation, 97 20, 2043Ibdah,J.A.; Tein,I.; Dionisi-Vici,C.; Bennett,M.J.; IJlst,L.; Gibson,B.; Wanders,R.J.; Strauss,A.W. 1998. Mild trifunctional protein deficiency is associated with progressive neuropathy and myopathy and suggests a novel genotype-phenotype correlation The Journal of clinical investigation, 102 6, 1193Khuchua,Z.A.; Qin,W.; Boero,J.; Cheng,J.; Payne,R.M.; Saks,V.A.; Strauss,A.W. 1998. Octamer formation and coupling of cardiac sarcomeric mitochondrial creatine kinase are mediated by charged N-terminal residues The Journal of biological chemistry, 273 36, 22990Qin,W.; Khuchua,Z.; Cheng,J.; Boero,J.; Payne,R.M.; Strauss,A.W. 1998. Molecular characterization of the creatine kinases and some historical perspectives Molecular and cellular biochemistry, 184 1-2, 153Strauss, Arnold W. 1998. The molecular basis of congenital cardiac disease. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual, 1 , 179-188Strauss,A.W. 1998. The molecular basis of congenital cardiac disease , 1 , 179Eder,M.; Krautle,F.; Dong,Y.; Vock,P.; Kieweg,V.; Kim,J.J.; Strauss,A.W.; Ghisla,S. 1997. Characterization of human and pig kidney long-chain-acyl-CoA dehydrogenases and their role in beta-oxidation European journal of biochemistry / FEBS, 245 3, 600Qin,W.; Khuchua,Z.; Klein,S.C.; Strauss,A.W. 1997. Elements regulating cardiomyocyte expression of the human sarcomeric mitochondrial creatine kinase gene in transgenic mice The Journal of biological chemistry, 272 40, 25210Zhang,Z.; Zhou,Y.; Mendelsohn,N.J.; Bauer,G.S.; Strauss,A.W. 1997. Regulation of the human long chain acyl-CoA dehydrogenase gene by nuclear hormone receptor transcription factors Biochimica et biophysica acta, 1350 1, 53Bromberg,B.I.; Mazziotti,M.V.; Canter,C.E.; Spray,T.L.; Strauss,A.W.; Foglia,R.P. 1996. Recognition and management of nonpenetrating cardiac trauma in children The Journal of pediatrics, 128 4, 536Isaacs,J.D.,Jr; Sims,H.F.; Powell,C.K.; Bennett,M.J.; Hale,D.E.; Treem,W.R.; Strauss,A.W. 1996. Maternal acute fatty liver of pregnancy associated with fetal trifunctional protein deficiency: molecular characterization of a novel maternal mutant allele Pediatric research, 40 3, 393Johnson,M.C.; Watson,M.S.; Strauss,A.W. 1996. Chromosome 22q11 monosomy and the genetic basis of congenital heart disease The Journal of pediatrics, 129 1, 1Strauss,A.W.; Johnson,M.C. 1996. The genetic basis of pediatric cardiovascular disease Seminars in perinatology, 20 6, 564Brackett,J.C.; Sims,H.F.; Rinaldo,P.; Shapiro,S.; Powell,C.K.; Bennett,M.J.; Strauss,A.W. 1995. Two alpha subunit donor splice site mutations cause human trifunctional protein deficiency The Journal of clinical investigation, 95 5, 2076Johnson,M.C.; Payne,R.M.; Grant,J.W.; Strauss,A.W. 1995. The genetic basis of paediatric heart disease Annals of Medicine, 27 3, 289Johnson,M.C.; Strauss,A.W.; Dowton,S.B.; Spray,T.L.; Huddleston,C.B.; Wood,M.K.; Slaugh,R.A.; Watson,M.S. 1995. Deletion within chromosome 22 is common in patients with absent pulmonary valve syndrome The American Journal of Cardiology, 76 1, 66Johnson,M.C.; Watson,M.S.; Strauss,A.W.; Spray,T.L. 1995. Anomalous origin of the right pulmonary artery from the aorta and CATCH 22 syndrome The Annals of Thoracic Surgery, 60 3, 681Leone,T.C.; Cresci,S.; Carter,M.E.; Zhang,Z.; Lala,D.S.; Strauss,A.W.; Kelly,D.P. 1995. The human medium chain Acyl-CoA dehydrogenase gene promoter consists of a complex arrangement of nuclear receptor response elements and Sp1 binding sites The Journal of biological chemistry, 270 27, 16308Leone,T.C.; Cresci,S.; Carter,M.E.; Zhang,Z.; Lala,D.S.; Strauss,A.W.; Kelly,D.P. 1995. The human medium chain acyl-CoA dehydrogenase gene promoter consists of a complex arrangement of nuclear receptor response elements and Sp1 binding sites The Journal of biological chemistry, 270 41, 24622Payne,R.M.; Johnson,M.C.; Grant,J.W.; Strauss,A.W. 1995. Toward a molecular understanding of congenital heart disease Circulation, 91 2, 494Peterson,K.L.; Sergienko,E.E.; Wu,Y.; Kumar,N.R.; Strauss,A.W.; Oleson,A.E.; Muhonen,W.W.; Shabb,J.B.; Srivastava,D.K. 1995. Recombinant human liver medium-chain acyl-CoA dehydrogenase: purification, characterization, and the mechanism of interactions with functionally diverse C8-CoA molecules Biochemistry, 34 45, 14942Sims,H.F.; Brackett,J.C.; Powell,C.K.; Treem,W.R.; Hale,D.E.; Bennett,M.J.; Gibson,B.; Shapiro,S.; Strauss,A.W. 1995. The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy Proceedings of the National Academy of Sciences of the United States of America, 92 3, 841Strauss,A.W.; Powell,C.K.; Hale,D.E.; Anderson,M.M.; Ahuja,A.; Brackett,J.C.; Sims,H.F. 1995. Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood Proceedings of the National Academy of Sciences of the United States of America, 92 23, 10496Weinberger,M.J.; Rinaldo,P.; Strauss,A.W.; Bennett,M.J. 1995. Intact alpha-subunit is required for membrane-binding of human mitochondrial trifunctional beta-oxidation protein, but is not necessary for conferring 3-ketoacyl-CoA thiolase activity to the beta-subunit Biochemical and biophysical research communications, 209 1, 47Ziadeh,R.; Hoffman,E.P.; Finegold,D.N.; Hoop,R.C.; Brackett,J.C.; Strauss,A.W.; Naylor,E.W. 1995. Medium chain acyl-CoA dehydrogenase deficiency in Pennsylvania: neonatal screening shows high incidence and unexpected mutation frequencies Pediatric research, 37 5, 675Brackett,J.C.; Sims,H.F.; Steiner,R.D.; Nunge,M.; Zimmerman,E.M.; deMartinville,B.; Rinaldo,P.; Slaugh,R.; Strauss,A.W. 1994. A novel mutation in medium chain acyl-CoA dehydrogenase causes sudden neonatal death The Journal of clinical investigation, 94 4, 1477Djordjevic,S.; Dong,Y.; Paschke,R.; Frerman,F.E.; Strauss,A.W.; Kim,J.J. 1994. Identification of the catalytic base in long chain acyl-CoA dehydrogenase Biochemistry, 33 14, 4258Johnson,M.C.; Strauss,A.W. 1994. The jury is still out regarding balloon therapy for native aortic coarctation Journal of the American College of Cardiology, 24 6, 1589Kelly,D.P.; Strauss,A.W. 1994. Inherited cardiomyopathies The New England journal of medicine, 330 13, 913O'Shea,D.L.; Gast,M.J.; Murdock,G.L.; Payne,R.M.; Strauss,A.W. 1994. Expression of engineered human 17 beta-estradiol dehydrogenase in a prokaryotic system Journal of the Society for Gynecologic Investigation, 1 2, 143Payne,R.M.; Strauss,A.W. 1994. Developmental expression of sarcomeric and ubiquitous mitochondrial creatine kinase is tissue-specific Biochimica et biophysica acta, 1219 1, 33Payne,R.M.; Strauss,A.W. 1994. Expression of the mitochondrial creatine kinase genes Molecular and cellular biochemistry, 133-134 , 235Andresen,B.S.; Kolvraa,S.; Bross,P.; Bolund,L.; Curtis,D.; Eiberg,H.; Zhang,Z.; Kelly,D.P.; Strauss,A.W.; Gregersen,N. 1993. A silent A to G mutation in exon 11 of the medium-chain acyl-CoA dehydrogenase (MCAD) gene Human molecular genetics, 2 4, 488Hainline,B.E.; Kahlenbeck,D.J.; Grant,J.; Strauss,A.W. 1993. Tissue specific and developmental expression of rat long-and medium-chain acyl-CoA dehydrogenases Biochimica et biophysica acta, 1216 3, 460Johnson,M.C.; Canter,C.E.; Strauss,A.W.; Spray,T.L. 1993. Repair of coarctation of the aorta in infancy: comparison of surgical and balloon angioplasty American Heart Journal, 125 2 Pt 1, 464Payne,R.M.; Friedman,D.L.; Grant,J.W.; Perryman,M.B.; Strauss,A.W. 1993. Creatine kinase isoenzymes are highly regulated during pregnancy in rat uterus and placenta The American Journal of Physiology, 265 4 Pt 1, E624Whelan,A.J.; Strauss,A.W.; Hale,D.E.; Mendelsohn,N.J.; Kelly,D.P. 1993. Expression and characterization of human mutant (glutamic acid304) medium-chain acyl-coenzyme A dehydrogenase in mammalian cells Pediatric research, 34 5, 694Zhang,Z.; Kolvraa,S.; Zhou,Y.; Kelly,D.P.; Gregersen,N.; Strauss,A.W. 1993. Three RFLPs defining a haplotype associated with the common mutation in human medium-chain acyl-CoA dehydrogenase (MCAD) deficiency occur in Alu repeats American Journal of Human Genetics, 52 6, 1111Ghisla,S.; Engst,S.; Moll,M.; Bross,P.; Strauss,A.W.; Kim,J.J. 1992. Alpha, beta-dehydrogenation by acyl-CoA dehydrogenases: role of functional groups at the active center Progress in clinical and biological research, 375 , 127Kelly,D.P.; Hale,D.E.; Rutledge,S.L.; Ogden,M.L.; Whelan,A.J.; Zhang,Z.; Strauss,A.W. 1992. Molecular basis of inherited medium-chain acyl-CoA dehydrogenase deficiency causing sudden child death Journal of inherited metabolic disease, 15 2, 171Kelly,D.P.; Whelan,A.J.; Hale,D.E.; Rinaldo,P.; Rutledge,S.L.; Zhang,Z.; Strauss,A.W. 1992. Molecular characterization of medium-chain acyl-CoA dehydrogenase deficiency causing sudden death Progress in clinical and biological research, 375 , 463Kelly,D.P.; Zhang,Z.; Raisher,B.D.; Ogden,M.L.; Strauss,A.W. 1992. Identification and characterization of the 5' regulatory region of the human medium-chain acyl-CoA dehydrogenase (MCAD) gene Progress in clinical and biological research, 375 , 143Raisher,B.D.; Grant,J.W.; Martin,T.C.; Strauss,A.W.; Spray,T.L. 1992. Complete repair of total anomalous pulmonary venous connection in infancy The Journal of thoracic and cardiovascular surgery, 104 2, 443Raisher,B.D.; Gulick,T.; Zhang,Z.; Strauss,A.W.; Moore,D.D.; Kelly,D.P. 1992. Identification of a novel retinoid-responsive element in the promoter region of the medium chain acyl-coenzyme A dehydrogenase gene The Journal of biological chemistry, 267 28, 20264Su,C.Y.; Payne,M.; Strauss,A.W.; Dillmann,W.H. 1992. Selective reduction of creatine kinase subunit mRNAs in striated muscle of diabetic rats The American Journal of Physiology, 263 2 Pt 1, E310Tanaka,K.; Yokota,I.; Coates,P.M.; Strauss,A.W.; Kelly,D.P.; Zhang,Z.; Gregersen,N.; Andresen,B.S.; Matsubara,Y.; Curtis,D. 1992. Mutations in the medium chain acyl-CoA dehydrogenase (MCAD) gene Human mutation, 1 4, 271Zhang,Z.F.; Kelly,D.P.; Kim,J.J.; Zhou,Y.Q.; Ogden,M.L.; Whelan,A.J.; Strauss,A.W. 1992. Structural organization and regulatory regions of the human medium-chain acyl-CoA dehydrogenase gene Biochemistry, 31 1, 81Fontanet,H.L.; Trask,R.V.; Haas,R.C.; Strauss,A.W.; Abendschein,D.R.; Billadello,J.J. 1991. Regulation of expression of M, B, and mitochondrial creatine kinase mRNAs in the left ventricle after pressure overload in rats Circulation research, 68 4, 1007Gregersen,N.; Andresen,B.S.; Bross,P.; Winter,V.; Rudiger,N.; Engst,S.; Christensen,E.; Kelly,D.; Strauss,A.W.; Kolvraa,S. 1991. Molecular characterization of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency: identification of a lys329 to glu mutation in the MCAD gene, and expression of inactive mutant enzyme protein in E. coli Human genetics, 86 6, 545Gregersen,N.; Andresen,B.S.; Bross,P.; Winter,V.; Rudiger,N.; Engst,S.; Ghisla,S.; Christensen,E.; Kelly,D.; Strauss,A.W. 1991. Characterization of a disease-causing Lys329 to Glu mutation in 16 patients with medium-chain acyl-CoA dehydrogenase deficiency Journal of inherited metabolic disease, 14 3, 314Klein,S.C.; Haas,R.C.; Perryman,M.B.; Billadello,J.J.; Strauss,A.W. 1991. Regulatory element analysis and structural characterization of the human sarcomeric mitochondrial creatine kinase gene The Journal of biological chemistry, 266 27, 18058Murphy,A.M.; Jones,L.,2nd; Sims,H.F.; Strauss,A.W. 1991. Molecular cloning of rat cardiac troponin I and analysis of troponin I isoform expression in developing rat heart Biochemistry, 30 3, 707Payne,R.M.; Haas,R.C.; Strauss,A.W. 1991. Structural characterization and tissue-specific expression of the mRNAs encoding isoenzymes from two rat mitochondrial creatine kinase genes Biochimica et biophysica acta, 1089 3, 352Shackelford,P.G.; Strauss,A.W. 1991. Kawasaki syndrome The New England journal of medicine, 324 23, 1664Agrawal,H.C.; Agrawal,D.; Strauss,A.W. 1990. Cleavage of the P0 glycoprotein of the rat peripheral nerve myelin: tentative identification of cleavage site and evidence for the precursor-product relationship Neurochemical research, 15 10, 993Bross,P.; Engst,S.; Strauss,A.W.; Kelly,D.P.; Rasched,I.; Ghisla,S. 1990. Characterization of wild-type and an active site mutant of human medium chain acyl-CoA dehydrogenase after expression in Escherichia coli The Journal of biological chemistry, 265 13, 7116Haas,R.C.; Strauss,A.W. 1990. Separate nuclear genes encode sarcomere-specific and ubiquitous human mitochondrial creatine kinase isoenzymes The Journal of biological chemistry, 265 12, 6921Kelly,D.P.; Strauss,A.W. 1990. The tissue-specific and developmental regulation of expression of rat medium-chain acyl-CoA dehydrogenase mRNA Progress in clinical and biological research, 321 , 599Kelly,D.P.; Whelan,A.J.; Ogden,M.L.; Alpers,R.; Zhang,Z.F.; Bellus,G.; Gregersen,N.; Dorland,L.; Strauss,A.W. 1990. Molecular characterization of inherited medium-chain acyl-CoA dehydrogenase deficiency Proceedings of the National Academy of Sciences of the United States of America, 87 23, 9236Long,C.A.; Bauer,G.S.; Lowe,M.E.; Strauss,A.W.; Gast,M.J. 1990. Isolation and characterization of the gene from a human genome encoding 17 beta-estradiol dehydrogenase: a comparison of Jar and BeWo choriocarcinoma cell lines American Journal of Obstetrics and Gynecology, 163 6 Pt 1, 1976Lowe,M.; Strauss,A.W.; Alpers,R.; Seetharam,S.; Alpers,D.H. 1990. Molecular cloning and expression of a cDNA encoding the membrane-associated rat intestinal alkaline phosphatase Biochimica et biophysica acta, 1037 2, 170Lowe,M.E.; Rosenblum,J.L.; McEwen,P.; Strauss,A.W. 1990. Cloning and characterization of the human colipase cDNA Biochemistry, 29 3, 823Lowe,M.E.; Strauss,A.W. 1990. Expression of a Nagao-type, phosphatidylinositol-glycan anchored alkaline phosphatase in human choriocarcinomas Cancer research, 50 13, 3956Strauss,A.W.; Duran,M.; Zhang,Z.F.; Alpers,R.; Kelly,D.P. 1990. Molecular analysis of medium chain acyl-CoA dehydrogenase deficiency Progress in clinical and biological research, 321 , 609Strauss,A.W.; Goldring,D. 1990. Coarctation of the aorta in infants Circulation, 82 5, 1882Billadello,J.J.; Fontanet,H.L.; Strauss,A.W.; Abendschein,D.R. 1989. Characterization of MB creatine kinase isoform conversion in vitro and in vivo in dogs The Journal of clinical investigation, 83 5, 1637Chu,T.W.; Eftime,R.; Sztul,E.; Strauss,A.W. 1989. Synthetic transit peptides inhibit import and processing of mitochondrial precursor proteins The Journal of biological chemistry, 264 16, 9552Gast,M.J.; Sims,H.F.; Murdock,G.L.; Gast,P.M.; Strauss,A.W. 1989. Isolation and sequencing of a complementary deoxyribonucleic acid clone encoding human placental 17 beta-estradiol dehydrogenase: identification of the putative cofactor binding site American Journal of Obstetrics and Gynecology, 161 6 Pt 1, 1726Haas,R.C.; Korenfeld,C.; Zhang,Z.F.; Perryman,B.; Roman,D.; Strauss,A.W. 1989. Isolation and characterization of the gene and cDNA encoding human mitochondrial creatine kinase The Journal of biological chemistry, 264 5, 2890Kelly,D.P.; Gordon,J.I.; Alpers,R.; Strauss,A.W. 1989. The tissue-specific expression and developmental regulation of two nuclear genes encoding rat mitochondrial proteins. Medium chain acyl-CoA dehydrogenase and mitochondrial malate dehydrogenase The Journal of biological chemistry, 264 32, 18921Lowe,M.E.; Rosenblum,J.L.; Strauss,A.W. 1989. Cloning and characterization of human pancreatic lipase cDNA The Journal of biological chemistry, 264 33, 20042Sztul,E.S.; Chu,T.W.; Strauss,A.W.; Rosenberg,L.E. 1989. Translocation of precursor proteins into the mitochondrial matrix occurs through an environment accessible to aqueous perturbants Journal of cell science, 94 ( Pt 4) Pt 4, 695Griffin,M.L.; Hernandez,A.; Martin,T.C.; Goldring,D.; Bolman,R.M.; Spray,T.L.; Strauss,A.W. 1988. Dilated cardiomyopathy in infants and children Journal of the American College of Cardiology, 11 1, 139Stallings,R.L.; Olson,E.; Strauss,A.W.; Thompson,L.H.; Bachinski,L.L.; Siciliano,M.J. 1988. Human creatine kinase genes on chromosomes 15 and 19, and proximity of the gene for the muscle form to the genes for apolipoprotein C2 and excision repair American Journal of Human Genetics, 43 2, 144Sztul,E.S.; Chu,T.W.; Strauss,A.W.; Rosenberg,L.E. 1988. Import of the malate dehydrogenase precursor by mitochondria. Cleavage within leader peptide by matrix protease leads to formation of intermediate-sized form The Journal of biological chemistry, 263 24, 12085Trask,R.V.; Strauss,A.W.; Billadello,J.J. 1988. Developmental regulation and tissue-specific expression of the human muscle creatine kinase gene The Journal of biological chemistry, 263 32, 17142Abendschein,D.R.; Serota,H.; Plummer,T.H.,Jr; Amiraian,K.; Strauss,A.W.; Sobel,B.E.; Jaffe,A.S. 1987. Conversion of MM creatine kinase isoforms in human plasma by carboxypeptidase N The Journal of laboratory and clinical medicine, 110 6, 798Chu,T.W.; Grant,P.M.; Strauss,A.W. 1987. Mutation of a neutral amino acid in the transit peptide of rat mitochondrial malate dehydrogenase abolishes binding and import The Journal of biological chemistry, 262 32, 15759Chu,T.W.; Grant,P.M.; Strauss,A.W. 1987. The role of arginine residues in the rat mitochondrial malate dehydrogenase transit peptide The Journal of biological chemistry, 262 26, 12806Grace,A.M.; Gualdoni,J.; Strauss,A.W.; Sobel,B.E. 1987. Quantification of isoforms of plasma MM creatine kinase (CK) with an immunoblot procedure Catheterization and cardiovascular diagnosis, 13 1, 26Grant,P.M.; Roderick,S.L.; Grant,G.A.; Banaszak,L.J.; Strauss,A.W. 1987. Comparison of the precursor and mature forms of rat heart mitochondrial malate dehydrogenase Biochemistry, 26 1, 128Hortin,G.; Fok,K.F.; Toren,P.C.; Strauss,A.W. 1987. Sulfation of a tyrosine residue in the plasmin-binding domain of alpha 2-antiplasmin The Journal of biological chemistry, 262 7, 3082Kelly,D.P.; Kim,J.J.; Billadello,J.J.; Hainline,B.E.; Chu,T.W.; Strauss,A.W. 1987. Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue Proceedings of the National Academy of Sciences of the United States of America, 84 12, 4068Seetharam,S.; Ovitt,C.; Strauss,A.W.; Rubin,D.; Alpers,D.H. 1987. Fat feeding stimulates only one of the two mRNAs encoding rat intestinal membranous and secreted alkaline phosphatase Biochemical and biophysical research communications, 145 1, 363Alpers,D.H.; Helms,D.; Seetharam,S.; May,V.L.; Strauss,A.W. 1986. In vitro translation of intestinal sucrase-isomaltase and glucoamylase Biochemical and biophysical research communications, 134 1, 37Billadello,J.J.; Kelly,D.P.; Roman,D.G.; Strauss,A.W. 1986. The complete nucleotide sequence of canine brain B creatine kinase mRNA: homology in the coding and 3' noncoding regions among species Biochemical and biophysical research communications, 138 1, 392Canter,C.E.; Martin,T.C.; Spray,T.L.; Weldon,C.S.; Strauss,A.W. 1986. Scimitar syndrome in childhood The American Journal of Cardiology, 58 7, 652Gordon,J.I.; Sims,H.F.; Strauss,A.W.; Scanu,A.M.; Edelstein,C.; Byrne,R.E. 1986. Proteolytic processing and compartmentalization of the primary translation products of mammalian apolipoprotein mRNAs CRC critical reviews in biochemistry, 20 1, 37Grant,P.M.; Tellam,J.; May,V.L.; Strauss,A.W. 1986. Isolation and nucleotide sequence of a cDNA clone encoding rat mitochondrial malate dehydrogenase Nucleic acids research, 14 15, 6053Hortin,G.; Chan,A.C.; Fok,K.F.; Strauss,A.W.; Atkinson,J.P. 1986. Sequence analysis of the COOH terminus of the alpha-chain of the fourth component of human complement. Identification of the site of its extracellular cleavage The Journal of biological chemistry, 261 19, 9065Hortin,G.; Folz,R.; Gordon,J.I.; Strauss,A.W. 1986. Characterization of sites of tyrosine sulfation in proteins and criteria for predicting their occurrence Biochemical and biophysical research communications, 141 1, 326Hortin,G.; Green,E.D.; Baenziger,J.U.; Strauss,A.W. 1986. Sulphation of proteins secreted by a human hepatoma-derived cell line. Sulphation of N-linked oligosaccharides on alpha 2HS-glycoprotein The Biochemical journal, 235 2, 407Hortin,G.; Sims,H.; Strauss,A.W. 1986. Identification of the site of sulfation of the fourth component of human complement The Journal of biological chemistry, 261 4, 1786Hortin,G.; Strauss,A.W. 1986. Effects of acidotropic compounds on the secretory pathway: inhibition of secretion and processing of the third and fourth components of complement Biochemical and biophysical research communications, 136 2, 603Hortin,G.; Tollefsen,D.M.; Strauss,A.W. 1986. Identification of two sites of sulfation of human heparin cofactor II The Journal of biological chemistry, 261 34, 15827Ovitt,C.E.; Strauss,A.W.; Alpers,D.H.; Chou,J.Y.; Boime,I. 1986. Expression of different-sized placental alkaline phosphatase mRNAs in placenta and choriocarcinoma cells Proceedings of the National Academy of Sciences of the United States of America, 83 11, 3781Billadello,J.J.; Roman,D.G.; Grace,A.M.; Sobel,B.E.; Strauss,A.W. 1985. The nature of post-translational formation of MM creatine kinase isoforms The Journal of biological chemistry, 260 28, 14988Gordon,J.I.; Sims,H.F.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1985. Extracellular processing of proapolipoprotein A-II in Hep G2 cell cultures is mediated by a 54-kDa protease immunologically related to cathepsin B The Journal of biological chemistry, 260 27, 14824Grace,A.M.; Strauss,A.W.; Sobel,B.E. 1985. Sensitive quantification of isoforms of canine MM creatine kinase with an immunoblot procedure suitable for large numbers of samples Analytical Biochemistry, 149 1, 209Hashimoto,H.; Abendschein,D.R.; Strauss,A.W.; Sobel,B.E. 1985. Early detection of myocardial infarction in conscious dogs by analysis of plasma MM creatine kinase isoforms Circulation, 71 2, 363Alpers,D.H.; Strauss,A.W.; Ockner,R.K.; Bass,N.M.; Gordon,J.I. 1984. Cloning of a cDNA encoding rat intestinal fatty acid binding protein Proceedings of the National Academy of Sciences of the United States of America, 81 2, 313Blaufuss,M.C.; Gordon,J.I.; Schonfeld,G.; Strauss,A.W.; Alpers,D.H. 1984. Biosynthesis of apolipoprotein C-III in rat liver and small intestinal mucosa The Journal of biological chemistry, 259 4, 2452Gordon,J.I.; Bisgaier,C.L.; Sims,H.F.; Sachdev,O.P.; Glickman,R.M.; Strauss,A.W. 1984. Biosynthesis of human preapolipoprotein A-IV The Journal of biological chemistry, 259 1, 468Gordon,J.I.; Sims,H.F.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1984. Human proapolipoprotein A-II is cleaved following secretion from Hep G2 cells by a thiol protease The Journal of biological chemistry, 259 24, 15556Hashimoto,H.; Grace,A.M.; Billadello,J.J.; Gross,R.W.; Strauss,A.W.; Sobel,B.E. 1984. Nondenaturing quantification of subforms of canine MM creatine kinase isoenzymes (isoforms) and their interconversion The Journal of laboratory and clinical medicine, 103 3, 470Leonard,W.J.; Strauss,A.W.; Go,M.F.; Alpers,D.H.; Gordon,J.I. 1984. Biosynthesis and compartmentalization of rat-intestinal vitamin-D-dependent calcium-binding protein European journal of biochemistry / FEBS, 139 3, 561Lowe,J.B.; Strauss,A.W.; Gordon,J.I. 1984. Expression of a mammalian fatty acid-binding protein in Escherichia coli The Journal of biological chemistry, 259 20, 12696Cockerham,J.T.; Martin,T.C.; Gutierrez,F.R.; Hartmann,A.F.,Jr; Goldring,D.; Strauss,A.W. 1983. Spontaneous closure of secundum atrial septal defect in infants and young children The American Journal of Cardiology, 52 10, 1267Edelstein,C.; Gordon,J.I.; Toscas,K.; Sims,H.F.; Strauss,A.W.; Scanu,A.M. 1983. In vitro conversion of proapoprotein A-I to apoprotein A-I. Partial characterization of an extracellular enzyme activity The Journal of biological chemistry, 258 19, 11430Gordon,J.I.; Alpers,D.H.; Ockner,R.K.; Strauss,A.W. 1983. The nucleotide sequence of rat liver fatty acid binding protein mRNA The Journal of biological chemistry, 258 5, 3356Gordon,J.I.; Budelier,K.A.; Sims,H.F.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1983. Biosynthesis of human preproapolipoprotein A-II The Journal of biological chemistry, 258 22, 14054Gordon,J.I.; Sims,H.F.; Lentz,S.R.; Edelstein,C.; Scanu,A.M.; Strauss,A.W. 1983. Proteolytic processing of human preproapolipoprotein A-I. A proposed defect in the conversion of pro A-I to A-I in Tangier's disease The Journal of biological chemistry, 258 6, 4037Perryman,M.B.; Strauss,A.W.; Buettner,T.L.; Roberts,R. 1983. Molecular heterogeneity of creatine kinase isoenzymes Biochimica et biophysica acta, 747 3, 284Perryman,M.B.; Strauss,A.W.; Olson,J.; Roberts,R. 1983. In vitro translation of canine mitochondrial creatine kinase messenger RNA Biochemical and biophysical research communications, 110 3, 967Gordon,J.I.; Smith,D.P.; Alpers,D.H.; Strauss,A.W. 1982. Cloning of a complementary deoxyribonucleic acid encoding a portion of rat intestinal preapolipoprotein AIV messenger ribonucleic acid Biochemistry, 21 22, 5424Gordon,J.I.; Smith,D.P.; Alpers,D.H.; Strauss,A.W. 1982. Proteolytic processing of the primary translation product of rat intestinal apolipoprotein A-IV mRNA. Comparison with preproapolipoprotein A-I processing The Journal of biological chemistry, 257 14, 8418Gordon,J.I.; Smith,D.P.; Andy,R.; Alpers,D.H.; Schonfeld,G.; Strauss,A.W. 1982. The primary translation product of rat intestinal apolipoprotein A-I mRNA is an unusual preproprotein The Journal of biological chemistry, 257 2, 971Graves,C.B.; Munns,T.W.; Willingham,A.K.; Strauss,A.W. 1982. Rat factor X is synthesized as a single chain precursor inducible by prothrombin fragments The Journal of biological chemistry, 257 21, 13108Strauss,A.W.; Boime,I. 1982. Compartmentation of newly synthesized proteins CRC critical reviews in biochemistry, 12 3, 205Taysi,K.; Strauss,A.W.; Yang,V.; Padmalatha,C.; Marshall,R.E. 1982. Terminal deletion of the long arm of chromosome 10 : q26 to qter. Case report and review of literature Annales de Genetique, 25 3, 141Canter,C.E.; Bower,R.J.; Strauss,A.W. 1981. Atypical Kawasaki disease with aortic aneurysm Pediatrics, 68 6, 885Graves,C.B.; Munns,T.W.; Carlisle,T.L.; Grant,G.A.; Strauss,A.W. 1981. Induction of prothrombin synthesis by prothrombin fragments Proceedings of the National Academy of Sciences of the United States of America, 78 8, 4772Henry,C.G.; Goldring,D.; Hartmann,A.F.; Weldon,C.S.; Strauss,A.W. 1981. Treatment of d-transposition of the great arteries: management of hypoxemia after balloon atrial septostomy The American Journal of Cardiology, 47 2, 299Henry,C.G.; Gutierrez,F.; Lee,J.T.; Hartmann,A.F.; Bell,M.J.; Bower,R.J.; Strauss,A.W. 1981. Aortic thrombosis presenting as congestive heart failure: an umbilical artery catheter complication The Journal of pediatrics, 98 5, 820Henry,C.G.; Strauss,A.W.; Keating,J.P.; Hillman,R.E. 1981. Congestive cardiomyopathy associated with beta-ketothiolase deficiency The Journal of pediatrics, 99 5, 754Mumford,R.A.; Pickett,C.B.; Zimmerman,M.; Strauss,A.W. 1981. Protease activities present in wheat germ and rabbit reticulocyte lysates Biochemical and biophysical research communications, 103 2, 565Mumford,R.A.; Pierzchala,P.A.; Strauss,A.W.; Zimmerman,M. 1981. Purification of a membrane-bound metalloendopeptidase from porcine kidney that degrades peptide hormones Proceedings of the National Academy of Sciences of the United States of America, 78 11, 6623Mumford,R.A.; Strauss,A.W.; Powers,J.C.; Pierzchala,P.A.; Nishino,N.; Zimmerman,M. 1980. A zinc metalloendopeptidase associated with dog pancreatic membranes The Journal of biological chemistry, 255 6, 2227Strauss,A.W.; Zimmerman,M.; Mumford,R.A.; Alberts,A.W. 1980. Processing of pre-proalbumin and pre-placental lactogen Annals of the New York Academy of Sciences, 343 , 168Zimmerman,M.; Ashe,B.M.; Alberts,A.W.; Pierzchala,P.A.; Powers,J.C.; Nishino,N.; Strauss,A.W.; Mumford,R.A. 1980. Protease activities present in dog pancreatic membranes that process human pre-placental lactogen Annals of the New York Academy of Sciences, 343 , 405Haymond,M.W.; Strauss,A.W.; Arnold,K.J.; Bier,D.M. 1979. Glucose homeostasis in children with severe cyanotic congenital heart disease The Journal of pediatrics, 95 2, 220Jones,L.A.; Jordan,D.K.; Taysi,K.; Strauss,A.W.; Toth,J.K. 1979. Partial duplication of the long arm of chromosome 5: a case due to balanced paternal translocation and review of the literature Human genetics, 51 1, 37Jones,L.A.; Taysi,K.; Strauss,A.W.; Hartmann,A.F. 1979. Partial trisomy 13 as a result of de novo (6p;13q) translocation Human genetics, 48 2, 245Strauss,A.W.; Zimmerman,M.; Boime,I.; Ashe,B.; Mumford,R.A.; Alberts,A.W. 1979. Characterization of an endopeptidase involved in pre-protein processing Proceedings of the National Academy of Sciences of the United States of America, 76 9, 4225Taysi,K.; Noetzel,M.J.; Strauss,A.W. 1979. Presumptive long arm deletion of chromosome 8: a new syndrome? Human genetics, 51 1, 49Hernandez,A.; Strauss,A.W.; McKnight,R.; Hartmann,A.F.,Jr. 1978. Diagnosis of pulmonary arteriovenous fistula by contrast echocardiography The Journal of pediatrics, 93 2, 258Strauss,A.W.; Bennett,C.A.; Donohue,A.M.; Rodkey,J.A.; Boime,I.; Alberts,A.W. 1978. Conversion of rat pre-proalbumin to proalbumin in vitro by ascites membranes. Demonstration by NH2-TERMINAL SEQUENCE ANALYSIS The Journal of biological chemistry, 253 17, 6270Strauss,A.W.; Bennett,C.D.; Donohue,A.M.; Rodkey,J.A.; Alberts,A.W. 1977. Rat liver pre-proalbumin: complete amino acid sequence of the pre-piece. Analysis of the direct translation product of albumin messenger RNA The Journal of biological chemistry, 252 19, 6846Strauss,A.W.; Donohue,A.M.; Bennett,C.D.; Rodkey,J.A.; Alberts,A.W. 1977. Rat liver preproalbumin: in vitro synthesis and partial amino acid sequence Proceedings of the National Academy of Sciences of the United States of America, 74 4, 1358Alberts,A.W.; Strauss,A.W.; Hennessy,S.; Vagelos,P.R. 1975. Regulation of synthesis of hepatic fatty acid synthetase: binding of fatty acid synthetase antibodies to polysomes Proceedings of the National Academy of Sciences of the United States of America, 72 10, 3956Alderson,P.O.; Jost,R.G.; Strauss,A.W.; Boonvisut,S.; Markham,J. 1975. Radionuclide angiocardiography. Improved diagnosis and quantitation of left-to-right shunts using area ratio techniques in children Circulation, 51 6, 1136Strauss,A.W.; Alberts,A.W.; Hennessy,S.; Vagelos,P.R. 1975. Regulation of synthesis of hepatic fatty acid synthetase: polysomal translation in a cell-free system Proceedings of the National Academy of Sciences of the United States of America, 72 11, 4366Strauss,A.W.; Santa-Maria,M.; Goldring,D. 1975. Constrictive pericarditis in children American Journal of Diseases of Children (1960), 129 7, 822Strauss,A.W.; Escobedo,M.; Goldring,D. 1974. Continuous monitoring of arterial oxygen tension in the newborn infant The Journal of pediatrics, 85 2, 254Strauss,A.W.; Goldring,D. 1974. Editorial: Valve replacement in acute rheumatic heart disease The Journal of pediatrics, 84 5, 786Strauss,A.W.; Goldring,D.; Kissane,J.; Hernandez,A.; Hartmann,A.F.; McKnight,C.R.; Weldon,C.S. 1974. Valve replacement in acute rheumatic heart disease The Journal of thoracic and cardiovascular surgery, 67 4, 659Keating,J.P.; Lell,M.E.; Strauss,A.W.; Zarkowsky,H.; Smith,G.E. 1973. Infantile methemoglobinemia caused by carrot juice The New England journal of medicine, 288 16, 824STRAUSS,A.W.; STRAUSS,A.W.,Jr. 1952. Pseudomucinous cystadenocarcinoma of the ovary American Journal of Obstetrics and Gynecology, 64 6, 1382Bennett, Michael J; Russell, Laurie K; Tokunaga, Chonan; Narayan, Srinivas B; Tan, Lu; Seegmiller, Adam; Boriack, Richard L; Strauss, Arnold W . Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase. Molecular genetics and metabolism, 89 1-2, 74-9