21-Hydroxylase Deficiency

21-hydroxylase deficiency is one among a group of problems known as congenital adrenal hyperplasias that impair hormone production and disrupt sexual improvement. 21-hydroxylase deficiency is liable for about ninety five percent of all instances of congenital adrenal hyperplasia. 17-Hydroxyprogesterone (17-OHP) may be converted to dihydrotestosterone (DHT) through an opportunity “backdoor” route that bypasses the traditional intermediates androstenedione and testosterone. on this backdoor pathway, 17-OHP is transformed to 5α-pregnane-threeα,17α-diol-20-one (pdiol), that's an wonderful substrate for the 17,20 lyase activity of CYP17A1 to provide androsterone.

Objective and Hypotheses:

The goal of this observes become to attain proof for the presence of the backdoor pathway in sufferers with 21-hydroxylase deficiency (21-OHD).

Author(s): Clemens Remer, Thomas Kamrath

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