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

Abstract

The conversion of 17-hydroxy-progesterone to 11-desoxycortisol and cortisol by normal human adrenal tissue, with a greater yield of the former, has been described. Adrenal glands from 2 normotensive cases of adrenogenital syndrome, due to congenital adrenocortical hyperplasia, failed to effect hydroxylation of 17-hydroxyprogesterone at either carbon 11 or 21. On the other hand, when 11-desoxycortisol was employed as a substance, both normal and abnormal adrenal glands were able to bring about a significant conversion to cortisol. A relatively large quantity of 17-hydroxyprogesterone (but no 21-desoxycortisol) was found in the 2 glands from patients with the adrenogenital syndrome, without prior addition of substrate or incubation. These data directly confirm earlier indirect work pointing to a lack of 21-hydroxylase", and the presence of "11-hydroxylase" in the adrenal glands of patients with the normotensive form of this disorder. The results also suggest that 21-hydroxylation occurs prior to 11-hydroxylation in man. It has also been indicated that postmortem adrenal tissue, obtained within 24 hours of death, is capable of accomplishing the described conversions to a limited degree.