In vitro steroidogenesis in testes of three infants, two with ambiguous external genitalia and one with true precocious puberty: Evidence for the presence of active 17 beta-hydroxysteroid oxidoreductase in immature human testes.
The present report investigated steroidogenesis in vitro in testis tissues obtained from two boys aged 8 months and 4 years with ambiguous external genitalia and male vagina, and a 4-year-old body with true precocious puberty. Histologically, testes of the former two boys are still immature and the testis of the last one contains differentiated Sertoli cells and primary spermatocytes, but no mature Leydig cells are recognized in any of them. In each testis, 17 beta-hydroxysteroid oxidoreductase is active for androstenedione in the presence of an excess amount of NADPH, while delta 5-3 beta-hydroxysteroid dehydrogenase and delta 4-steroid 5 alpha-reductase activities are limited. 17 alpha-Hydroxylase and C17--20 lyase are significantly active in each testis and are enhanced in the testis of the boy with precocious puberty. Although the testis tissue used in the present study may not be biologically normal and the number of cases investigated is still limited, the above results indicate that active 17 beta-hydroxysteroid oxidoreductase is present in immature human testes and that delta 5-3 beta-hydroxysteroid dehydrogenase may become active in the human testis at the advanced stage of the development of testicular function during the puberty.
D015067:17-Hydroxysteroid Dehydrogenases / Q000378:metabolism*; D002675:Child, Preschool; D012734:Disorders of Sex Development / Q000378:metabolism*; D006801:Humans; D066298:In Vitro Techniques; D007223:Infant; D008297:Male; D011629:Puberty, Precocious / Q000378:metabolism*; D013737:Testis / Q000378:metabolism* / Q000473:pathology; D013739:Testosterone / Q000096:biosynthesis*
D013739:Testosterone; D015067:17-Hydroxysteroid Dehydrogenases