Abstract
The case of a true hermaphrodite, with a normal ovary and an ovotestis is presented. The ovotestis was removed and incubated in vitro with tritiated steroids (testosterone, dehydroepiandrosterone, pregnenolone and 17 alpha-hydroxyprogesterone). Labeled metabolites were isolated and identified. Based upon these findings, a pathway of steroid biogenesis in this abnormal gonadal tissue is suggested. The ovotestis studied did not contain all the enzymes involved in ovarian steroidogenesis: 3 beta-hydroxysteroid dehydrogenase, isomerase, 17--20 desmolase and 17 beta-hydroxysteroid dehydrogenase were present, but other important enzymes, such as 16 and 17-hydroxylases, and aromatizing enzyme systems, were deficient or absent.
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