In Vitro Hydroxylation of Steroids by Whole Adrenal Homogenates of Beef, Normal Man, and Patients with the Adrenogenital Syndrome1

The partial or complete failure of the adrenal cortex to realize the biosynthesis of zA4-pregnene- 11,3, 17c, 21-triol-3, 20-dione (compound F) represents the basic defect in patients with the adrenogenital syndrome due to congential adreno- cortical hyperplasia (1-5). The most common form of this disorder (with normal blood pres- sure and without undue salt loss) has been in- directly attributed to a deficiency of adrenal "21- hydroxylase" as witnessed by the large quantities of urinary 3a, 17a, 20a-pregnanetriol (hereafter designated "pregnanetriol") and the relatively small amounts of steroids with an oxygen func- tion at carbon 21 (4, 6, 7).