Extraadrenal steroid 21-hydroxylase activity in a woman with congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency.
The 21-hydroxylation of plasma progesterone (P) has been demonstrated in pregnant, nonpregnant, and adrenalectomized women and in men. The fractional conversion of plasma progesterone to deoxycorticosterone (DOC), [rho]P-DOC BU, among those subjects was 0.009 +/- 0.001 (mean +/- SEM, n = 32). The [rho]P-DOC BU in a woman with congenital adrenal hyperplasia due to apparent adrenal steroid 21-hydroxylase deficiency was 0.010 when she was taking cortisone acetate, and the [rho]P-DOC BU determined when she was not taking cortisone acetate was 0.012. Moreover, the value computed for the fractional conversion of 17 alpha-hydroxyprogesterone to 11-deoxycortisol in this woman (0.004) was similar to that observed in a woman with normal adrenal function (0.005). Therefore, extraadrenal 21-hydroxylase activity in a woman with nonsalt-losing congenital adrenal hyperplasia due to 21-hydroxylase deficiency was similar to that found in persons with normal adrenal function.
D000311:Adrenal Glands / Q000201:enzymology; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy / Q000201:enzymology*; D000328:Adult; D003348:Cortisone / Q000031:analogs & derivatives / Q000627:therapeutic use; D003350:Cortodoxone / Q000097:blood; D003900:Desoxycorticosterone / Q000097:blood; D005260:Female; D006801:Humans; D006908:Hydroxyprogesterones / Q000097:blood; D008875:Middle Aged; D011374:Progesterone / Q000097:blood; D013255:Steroid 21-Hydroxylase / Q000378:metabolism*; D013250:Steroid Hydroxylases / Q000172:deficiency* / Q000378:metabolism*
D006908:Hydroxyprogesterones; D003900:Desoxycorticosterone; D011374:Progesterone; D013250:Steroid Hydroxylases; D013255:Steroid 21-Hydroxylase; D003348:Cortisone; D003350:Cortodoxone