BIOSYNTHESIS OF PREGNANETRIOLONE AND PREGNANETETROL IN CONGENITAL ADRENAL HYPERPLASIA

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Abstract
Following the simultaneous intravenous injection of 17[alpha]-hydroxypregnenolone-3H and 17[alpha]-hydroxyprogesterone-14C to a patient with congenital adrenal hyperplasia, the following labelled metabolites were isolated from the urine: 17[alpha]-OH-pregnanolone, 5[beta]-pregnane-3[alpha],17[alpha],. 20[alpha]-triol, pregn-5-ene-3[beta],17[alpha],20[alpha]-triol, pregnanetriolone (3[alpha],17[alpha], 20[alpha]-trihydroxy-5[beta]-pregnan-ll-one) and pregnanetetrol (5[beta]-pregnane-3[alpha],11[beta],17[alpha],20[alpha]-tetrol). The 3H/14C ratio recovered in the urinary pregnanetriolone and pregnanetetrol was higher than in the injected mixture. It thus follows that 17[alpha]-hydroxypregnenolone-3H was converted to both metabolites, at least in part, bu a pathway(s) not including 17[alpha]-hydroxyprogesterone. Two additional pathways have been considered, I: 17[alpha]-OH-pregnenolone[forward arrow]pregn-5-ene-3[beta], 11[beta],17[alpha]-triol-20-one[forward arrow]21-deoxycortisol[forward arrow] pregnanetriolone (or tet-rol ), and II: 17[alpha]-OH-pregnenolone[forward arrow]pregn-5-ene-3[beta],ll[beta],17[alpha]-triol-20-one[forward arrow]pregn-5-enetriolone[forward arrow]pregnanetriolone (or tetrol ).