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Altered steroidogenesis in 21OHD. The block at CYP21A2 (bold X) and raised ACTH cause 21-deoxysteroids to accumulate and to undergo conversion to androgens and estrogens (darkened lines), as well as significant amounts of 21-deoxycortisol due to conversion of 17OHP by CYP11B1, a reaction that is insignificant in the presence of a functional CYP21A2 enzyme. Some 11-deoxycorticosterone is formed by peripheral conversion of P4 by hepatic cytochrome P450 (CYP) enzymes.
