Disease relevance of MLS000028688

• Serum 21-Deoxycortisol, 17-Hydroxyprogesterone, and 11-deoxycortisol in classic congenital adrenal hyperplasia: clinical and hormonal correlations and identification of patients with 11beta-hydroxylase deficiency among a large group with alleged 21-hydroxylase deficiency [1].
• The study was aimed at evaluation of usefulness of determination of blood serum 21-deoxycortisol concentration for the detection of heterozygous carriers of incomplete block of 21-hydroxylase synthesis leading to adrenal hyperplasia [2].

High impact information on MLS000028688

• The results indicated that P-450sccII was highly active in catalyzing hydroxylation of 11 beta-hydroxyprogesterone at the 17 alpha-position to give 21-deoxycortisol and cleavage of 17 alpha-hydroxyprogesterone at the 17-20 bond to give androstenedione with turnover numbers of 25 and 30 mol/min X mol of P-450, respectively [3].
• We studied the diagnostic value of the metabolite of 21-deoxycortisol (pregnanetriolone, Ptl) and the metabolite of 17OHP (pregnanetriol, PT) in identifying 21OHD in term and preterm neonates with elevated blood 17OHP on the newborn screening [4].
• A 4-fold elevation in plasma 21-deoxycortisol compared with a 200-fold elevation in 17OHP suggested that the elevated 17OHP derived from the normal pathway of testosterone synthesis in the testis [5].
• 11-Deoxycortisol and 21-deoxycortisol showed patterns similar to DOC, with a return to normal ACTH responses on LSD-DEX [6].
• Concentrations of unconjugated cortisol, 11-desoxycortisol, and 21-desoxycortisol were measured by radioimmunoassay in amniotic fluid throughout gestation [7].

Chemical compound and disease context of MLS000028688

• These normal values provide a basis for the application of assays of 11-desoxycortisol and 21-desoxycortisol in amniotic fluid in the prenatal diagnosis of congenital adrenal hyperplasia [7].
• Serum 21-deoxycortisol and 17-hydroxypregnenolone in parents of patients with congenital adrenal hyperplasia [8].
• Urinary 21-deoxycortisol and 17-hydroxyprogesterone in congenital adrenal hyperplasia [9].

Biological context of MLS000028688

• Analysis of 21-deoxycortisol, a marker of congenital adrenal hyperplasia, in blood by atmospheric pressure chemical ionization and electrospray ionization using multiple reaction monitoring [10].
• The antiserum was highly specific for cortisol with only corticosterone, cortisone, 11-deoxycortisol and 21-deoxycortisol showing significant cross-reaction (12.4, 6.6, 3.8 and 3.7%, respectively) [11].

Anatomical context of MLS000028688

• Cortisol, 11-desoxycortisol, and 21-desoxycortisol concentrations in amniotic fluid during normal pregnancy [7].
• Competition studies with [3H]TA-labeled eel gill cytosol and radioinert steroids gave the following binding hierarchy: TA greater than dexamethasone greater than cortisol greater than 11-deoxycortisol greater than 21-deoxycortisol [12].
• The mitochondria, prepared from pooled, frozen, polycystic ovarian tissue of 5 patients, converted [7-3H]17alpha-hydroxypregnenolone to 3beta, 11beta, 17alpha--trihydroxy-5-pregnen-20-one (yield 0.065%) and to 3beta, 17alpha-dihydroxy-5-pregnene-11,20-dione (0.22%), while [4-14C]17alpha-hydroxyprogesterone was converted to 21-deoxycortisol (0.1%) [13].
• Hormonal measurement of 17-hydroxyprogesterone, androstenedione, testosterone and 21-deoxycortisol and HLA typing and DNA analysis for 21-OH/C4/HLA class I and II genes in chorionic villus cells and amniocytes are utilized for prenatal diagnosis [14].

Associations of MLS000028688 with other chemical compounds

• Based upon the use of a specific radioimmunoassay method, the plasma levels of 21-deoxycortisol (21-DF) as well as of 17-hydroxyprogesterone (17- OHP) were examined in women with polycystic ovary disease - in normal conditions and after stimulation with corticotropin and chorionic gonadotrophin [15].
• The radioimmunoassay was used to measure 21-deoxycortisol in the plasma of normal subjects and patients with androgen excess [16].

Gene context of MLS000028688

• The application of a new highly-sensitive radioimmunoassay for plasma 21-deoxycortisol to the detection of steroid-21-hydroxylase deficiency [17].
• Mitochondria obtained from fresh, polycystic ovarian tissue of a single patient with POS converted [7-3H]17alpha-hydroxypregnenolone to 3beta,17alpha-dihydroxy-5-pregnene-11,20-dione (2.1%) and [4-14C]17alpha-hydroxyprogesterone to 21-deoxycortisol (0.1%) [13].

Analytical, diagnostic and therapeutic context of MLS000028688

• Plasma 21-deoxycortisol: comparison of a time-resolved fluoroimmunoassay using a biotinylated tracer with a radioimmunossay using (125)iodine [18].
• An enzyme immunoassay of 21-deoxycortisol (21-DOF) in plasma and dried blood spotted on filter paper has been developed [19].
• Using a highly specific radioimmunoassay recently described, plasma 21-deoxycortisol levels were measured in 55 heterozygous carriers of 21-hydroxylase deficiency (as demonstrated by HLA typing) [20].
• Unlike 17-hydroxyprogesterone, 21-deoxycortisol levels were significantly higher both in women (20.5 +/- 12.6 ng/dl) and in men (21.2 +/- 14.4 ng/l) suspected of being heterozygous carriers, both before and after stimulation with ACTH, as compared to those in the control group (6.9 +/- 3.6 in women and 7.9 +/- 2.8 in men) [2].

References