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©2013 UpToDate ® Print Email Synthetic defect in CYP11B1 (11-beta-hydroxylase) deficiency Pathways of adrenal steroid synthesis. A synthetic defect in 11-beta-hydroxylase leads to diminished cortisol synthesis, increased release of ACTH, and accumulation of 11- deoxycorticosterone (leading to mineralocorticoid excess), 11-deoxycortisol, and DHEA, androstenedione, and testosterone (leading to virilization). The numbers at the arrows refer to specific enzymes: 17 α = 17α-hydroxylase (P450c17); 17,20 = 17,20 lyase which is part of the P450c17 enzyme; 3§ = 3§-hydroxysteroid dehydrogenase; 21 = 21-hydroxylase (P450c21); 11β = 11β-hydroxylase; (P450c11); 18 refers to the two-step process of aldosterone synthase (P450c11as), resulting in the addition of an hydroxyl group that is then oxidized to an aldehyde group at the 18-carbon position; ? = unclear if pathway functions in vivo; DHEA = dehydroepiandrostenedione; 17KSR = 17-ketosteroid reductase; and A = aromatase.