3-beta-HSD (or 3β-hydroxysteroid dehydrogenase-isomerase) is an enzyme which catalyses the synthesis of progesterone from pregnenolone, 17-hydroxyprogesterone from 17-hydroxypregnenolone, and androstenedione from dehydroepiandrosterone in the adrenal gland. It is the only enzyme in the adrenal steroidogenic pathway that is not a member of the Cytochrome P450 family.
It is inhibited by trilostane.
Pathology
3-beta-HSD is inactivated in congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency (MIM.201810).
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