to P450scc the process of electrons transfer is leaky leading to superoxide production. The rate of electron leakage during metabolism depends on the functional groups of the steroid substrate. Regulation The expression of the enzyme in adrenocortical cells is regulated by the trophic hormone corticotropin (ACTH). Clinical significance A mutation in genes encoding 11β-hydroxylase is associated with congenital adrenal hyperplasia due to 11β-hydroxylase deficiency. 11β-hydroxylase is involved in the metabolism of 17α-hydroxyprogesterone to 21-deoxycortisol, in cases of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. See also Steroidogenic enzyme Additional images References Further reading External links Enzymes Metabolism