| 17,20-lyase deficiency |
a disorder of steroidogenesis due to deficiency of 17α-hydroxyprogesterone aldolase, an enzyme activity normally catalyzing the conversion of C21 to C9 steroid hormones (androgens and estrogens); the result is a type of congenital adrenal hyperplasia (type V). Males are pseudohermaphroditic and both sexes remain sexually infantile. The enzyme activity is part of the enzyme steroid 17α-monooxygenase; see also 17α-hydroxylase deficiency and see table at hyperplasia.
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