3-ketosteroid isomerase

Dehydroepiandrosterone comes into subsequent oxidative transformation with production of 16Alpha-hydroxydehydroepiandrosterone . This oxidation is catalyzed by Cytochrome P450, family 3, subfamily A, polypeptide 7 ( CYP3A7) [10] , [11] , [12] and Cytochrome P450, family 3, subfamily A, polypeptide 4 ( CYP3A4) [13] , [10] , [11] . Oxidative metabolite of this reaction as well as Dehydroepiandrosterone can be further sulfated by steroid sulfatase (microsomal), isozyme S ( STS ) [14] , [15] , [16] , [15] , [14] . Dehydroepiandrosterone and Dehydroepiandrosterone sulfate can be transformed into other compounds with hormonal activity, Androstendiol and Androstendiol sulfate , respectively. These two reactions are catalyzed by Hydroxysteroid (17-beta) dehydrogenase 1 ( HSD17B1) [17] , [18] , [17] , [18] , Hydroxysteroid (17-beta) dehydrogenase 2 ( HSD17B2) [19] , [20] , [20] , [21] , and Hydroxysteroid (17-beta) dehydrogenase 7 ( HSD17B7 ) [22] , [23] , [23] , [24] .

The fetal adrenal cortex lacks expression of the enzyme early on, thus mineralocorticoids (. aldosterone ) and glucocorticoids (. cortisol ) cannot be synthesized. This is significant because cortisol induces type II pneumocytes of the lungs to synthesize and secrete pulmonary surfactant ; without pulmonary surfactant to reduce the alveolar surface tension , premature neonates may die of neonatal respiratory distress syndrome . If delivery is unavoidable (. because of placental abruption , or pre-eclampsia / HELLP syndrome ), then glucocorticoids (. cortisol) can be administered.

The second isoenzyme of 5α reductase is deficient in the classic intersex condition ( pseudovaginal perineoscrotal hypospadias ), or 5α-reductase deficiency . It was first discovered in indigenous cultures of Papua, New Guinea , where children were born with feminine genitalia in the absence of endogenous DHT during pregnancy, but with the surge of testosterone during adolescence, changed to males at puberty. Because of this change at puberty, the condition is also sometimes called " guevedoche ." [24] There is a range of external appearance that has been described of external genitalia at birth, with varying degrees of virilization.

3-ketosteroid isomerase

3-ketosteroid isomerase

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