Accession NumberHMDB04030
Common_Name21-Deoxycortisol
DescriptionPlasma 21-deoxycortisol (21DF) is an excellent marker of 21-hydroxylase deficiency. Currently, it is the only marker able to detect heterozygous carriers with 21-hydroxylase deficiency after Adrenocorticotropic Hormone (ACTH) stimulation. The syndrome of congenital adrenal hyperplasia (CAH) comprises the spectrum of autosomal recessive enzymatic disorders that impair cortisol biosynthesis. The hormonal pattern and clinical manifestations result from hyperstimulation of the adrenal cortex by excessive production of ACTH, untied from the negative feedback exerted by reduced cortisol levels, and the ultimate accumulation of F precursors and androgens. These abnormalities predispose the female newborn to ambiguous genitalia (female pseudohermaphroditism) and precocious puberty that may occur in both sexes. CAH due to 21-hydroxylase deficiency (21OHD) comprises nearly 90% of all cases, with an estimated worldwide incidence of 1 in 14,000 live births. Because 21-deoxycortisol (21DF) is an 11b-hydroxylase (11bOH) derivative of 17-hydroxyprogesterone (17OHP), its serum levels are parallel and proportionally elevated in patients with 21OHD but decreased or undetectable in those with 11b-hydroxylase deficiency (11bOHD). Due to the marked buildup of 17OHP in 21OHD, this precursor steroid can proceed directly to 11-hydroxylation, producing distinct elevations of 21DF. (PMID: 16551734, 10731638)
Chemical_IUPAC_Name(8S,9S,10R,13S,14S,17R)-17-acetyl-11,17-dihydroxy-10,13-dimethyl-2,6,7,8,9,11,12,14,15,16-decahydro-1H-cyclopenta[a]phenanthren-3-one
Chemical FormulaC21H30O4
Sample ConcentrationNot Available
Mass ValueNot Available
Mass UnitNot Available
ManufacturerNot Available
AnalyzerNot Available
DeliveryNot Available
IonizationNot Available
Predicted 1H NMR SpectrumDownload
Predicted 1H NMR PeaklistDownload
Predicted 13C NMR SpectrumDownload
Predicted 13C NMR PeaklistDownload
Sample ConcentrationNot Available
Mass ValueNot Available
Mass UnitNot Available
ManufacturerNot Available
FrequencyNot Available
1H NMR SpectrumNot Available
Sample ConcentrationNot Available
Mass ValueNot Available
Mass UnitNot Available
ManufacturerNot Available
FrequencyNot Available
13C NMR SpectrumNot Available
Low Energy VoltageNot Available
Low Energy SpectrumNot Available
Low Energy PeaklistNot Available
Medium Energy SpectrumNot Available
Hight Energy SpectrumNot Available