1.3.1 Compartmentalization and Regulation Introduction (Diagram)
-Where do enzymes catalyze reactions within steroidogenic cells?
-How is cytoplasmic cholesterol transferred from the outer to inner
mitochondrial membrane?
-What is the rate-limiting step in steroid hormone biosynthesis?
The
enzymes catalyzing specific reactions within steroidogenic cells are
localized in the membranes of either the mitochondria or the smooth
endoplasmic reticulum or SER.
Specific enzymatic reactions occur because steroid intermediates
diffuse between these two compartments as well as out of the cells.
In the case of estrogen biosynthesis, the reaction sequence occurs in
two different cell types as well as two sub-cellular compartments.
Cytoplasmic
cholesterol is transferred from the outer mitochondrial membrane to the
inner mitochondrial membrane by steroid acute regulatory protein or
StAR.
Transfer of cholesterol from the outer to inner mitochondrial membrane
is the rate limiting step in steroid hormone biosynthesis. Mutations in the StAR gene cause the potentially lethal disease, lipid congenital adrenal hyperplasia.
In
this condition, the cell fills with cholesterol and cholesterol esters
because transfer across the mitochondrial membrane is blocked.