Cytochrome P450 11B1 (None)

Cytochrome P450 11B1 (CYP11B1), also known as steroid 11β-hydroxylase, is a mitochondrial enzyme that belongs to the cytochrome P450 superfamily. It plays a critical role in the biosynthesis of glucocorticoids, particularly cortisol and corticosterone, within the adrenal cortex. It catalyzes the final steps in cortisol and corticosterone synthesis: Converts 11-deoxycortisol to cortisol and converts 11-deoxycorticosterone to corticosterone. Mutations in CYP11B1 cause congenital adrenal hyperplasia (CAH) due to 11β-hydroxylase deficiency, leading to excess androgen production and virilization. Overactivity or dysregulation can contribute to Cushing's disease through excessive cortisol production. The closest paralog is CYP11B2 (aldosterone synthase), which primarily produces aldosterone rather than cortisol/corticosterone but shares significant sequence similarity and overlapping substrate specificity.

Other names

Steroid 11β-hydroxylaseCYP11B1, mitochondrial

Inhibition of 11β-hydroxylation of steroid substrates