Accumulation of these substrates, pregnenolone and progesterone will enhance the 21-hydroxylation and 11 [beta]-hydroxylation steps in the mineralocorticoid pathway resulting in an overproduction of 11-deoxycorticosterone
Most important are the pathways through which orally administered progesterone is transformed into 11-deoxycorticosterone
(DOC) and further into the metabolites that form pregnanolone (3[alpha],-hydroxy-5[alpha]-pregnane-20-one) and its isomer allopregnanolone (3[beta]-20[beta]-dihydroxy-5[alpha]-pregnane).
As measured by HPLC, corticosterone and 11-deoxycorticosterone
secretion to the medium increased linearly for at least 24 hr.
We have tentatively identified 11-deoxycorticosterone
, androstadienedione, and androstenedione in the effluent.
Deficiency of 21-hydroxylase prevents the conversion of 17-hydroxyprogesterone to 11-deoxycorticosterone
, leading to excessive production of androgens, which ultimately affects several stages of growth and development (4).
Simultaneous radioimmunoassay of plasma aldosterone, corticosterone, 11-deoxycorticosterone
, progesterone, 17-hydroxyprogesterone, 11-deoxycortisol, cortisol and cortisone.
Deficiency in the CYP21 gene prevents the conversion of 17-hydroxyprogesterone to 11-deoxycorticosterone
, leading to an excessive production of androgen, which in turn affects several stages of growth and development (1).