1.02011-09-21 00:27:30 UTC2015-07-21 06:57:37 UTCFDB02328411beta-Hydroxyprogesterone11beta-Hydroxyprogesterone is a normal human metabolite. Plasma 11beta-Hydroxyprogesterone concentrations does not vary significantly as a function of age, sex, or phase of the menstrual cycle, in contrast to 17-hydroxyprogesterone. Increased plasma 11beta-Hydroxyprogesterone levels in late-onset adrenal 21-hydroxylase deficiency suggest a mild defect of the mineralocorticoid pathway. 21-hydroxylase deficiency (OMIM 201910) is probably the most frequent (if not the most frequent) autosomal recessive genetic disease, occurring in almost 1% of Caucasians and about 3% of Ashkenazi Jews. 21-hydroxylase deficiency is unusual among genetic diseases in that approximately 95% of the mutant alleles have apparently been generated by recombination between a normally active gene (CYP21) and a closely linked pseudogene (CYP21P). There are 4 recognized clinical forms of congenital adrenal hyperplasia, the majority of cases being associated with 21-hydroxylase deficiency: salt-wasting (SW), simple virilizing (SV), nonclassic (NC) late-onset (also called attenuated and acquired), and cryptic. (PMID: 3546944, 2537337) [HMDB]
11beta-hydroxyprogesterone acts as a mineralocorticoid agonist in stimulating Na+ absorption in mammalian principal cortical collecting duct cells. 11 beta-hydroxyprogesterone (11OHP) activated the transiently expressed hMR in COS-7 cells in a dose-dependent manner (ED(50): 10(-8) M) and, like aldosterone, stimulated Ams I(sc) in mpkCCD(cl4) cells. Docking 11OHP within the hMR-ligand-binding domain homology model revealed that the agonist activity of 11OHP is caused by contacts between its 11 beta-hydroxyl group and Asn770. Furthermore, 11OHP was unable to activate the mutant hMR/N770A, in which Ala is substituted for Asn at position 770. These findings demonstrate that in the absence of the 21-hydroxyl group, the 11 beta-hydroxyl group can produce the contact with the hMR-Asn770 required for the hMR activation leading to stimulated Na(+) absorption. [HMDB](11beta)-11-hydroxypregn-4-ene-3,20-dione11-beta-Hydroxypregn-4-ene-3,20-dione11-beta-Hydroxyprogesterone11b-Hydroxyprogesterone11beta-hydroxypregn-4-ene-3,20-dione21-DeoxycorticosteroneC21H30O3330.4611330.219494826(1S,2R,10S,11S,14S,15S,17S)-14-acetyl-17-hydroxy-2,15-dimethyltetracyclo[8.7.0.0^{2,7}.0^{11,15}]heptadec-6-en-5-one(1S,2R,10S,11S,14S,15S,17S)-14-acetyl-17-hydroxy-2,15-dimethyltetracyclo[8.7.0.0^{2,7}.0^{11,15}]heptadec-6-en-5-one600-57-7[H][C@@]12CC[C@H](C(C)=O)[C@@]1(C)C[C@H](O)[C@@]1([H])[C@@]2([H])CCC2=CC(=O)CC[C@]12CInChI=1S/C21H30O3/c1-12(22)16-6-7-17-15-5-4-13-10-14(23)8-9-20(13,2)19(15)18(24)11-21(16,17)3/h10,15-19,24H,4-9,11H2,1-3H3/t15-,16+,17-,18-,19+,20-,21+/m0/s1BFZHCUBIASXHPK-ATWVFEABSA-N belongs to the class of organic compounds known as gluco/mineralocorticoids, progestogins and derivatives. These are steroids with a structure based on a hydroxylated prostane moiety.Gluco/mineralocorticoids, progestogins and derivativesOrganic compoundsLipids and lipid-like moleculesSteroids and steroid derivativesPregnane steroidsAliphatic homopolycyclic compounds11-beta-hydroxysteroids20-oxosteroids3-oxo delta-4-steroidsCyclic alcohols and derivativesCyclohexenonesDelta-4-steroidsHydrocarbon derivativesOrganic oxidesSecondary alcohols11-beta-hydroxysteroid11-hydroxysteroid20-oxosteroid3-oxo-delta-4-steroid3-oxosteroidAlcoholAliphatic homopolycyclic compoundCarbonyl groupCyclic alcoholCyclic ketoneCyclohexenoneDelta-4-steroidHydrocarbon derivativeHydroxysteroidKetoneOrganic oxideOrganic oxygen compoundOrganooxygen compoundOxosteroidProgestogin-skeletonSecondary alcohol11beta-hydroxy steroidC21 steroids (gluco/mineralocorticoids, progestogins) and derivativesSolidlogp2.65logs-3.97solubility3.56e-02 g/llogp2.84pka_strongest_acidic18.88pka_strongest_basic-0.26iupac(1S,2R,10S,11S,14S,15S,17S)-14-acetyl-17-hydroxy-2,15-dimethyltetracyclo[8.7.0.0^{2,7}.0^{11,15}]heptadec-6-en-5-oneaverage_mass330.4611mono_mass330.219494826smiles[H][C@@]12CC[C@H](C(C)=O)[C@@]1(C)C[C@H](O)[C@@]1([H])[C@@]2([H])CCC2=CC(=O)CC[C@]12CformulaC21H30O3inchiInChI=1S/C21H30O3/c1-12(22)16-6-7-17-15-5-4-13-10-14(23)8-9-20(13,2)19(15)18(24)11-21(16,17)3/h10,15-19,24H,4-9,11H2,1-3H3/t15-,16+,17-,18-,19+,20-,21+/m0/s1inchikeyBFZHCUBIASXHPK-ATWVFEABSA-Npolar_surface_area54.37refractivity94.3polarizability38.12rotatable_bond_count1acceptor_count3donor_count1physiological_charge0formal_charge0SteroidogenesisSMP00130map00140Specdb::MsMs2329998Specdb::MsMs2329999Specdb::MsMs2330000Specdb::MsMs2635144Specdb::MsMs2635145Specdb::MsMs2635146Specdb::CMs2247Specdb::CMs2252Specdb::CMs169081HMDB0403128247#<Reference:0x0000555675d47148>#<Reference:0x0000555675d46ea0>#<Reference:0x0000555675d46c70>#<Reference:0x0000555675d46a68>#<Reference:0x0000555675d46888>#<Reference:0x0000555675d466a8>#<Reference:0x0000555675d464a0>#<Reference:0x0000555675d46298>#<Reference:0x0000555675d46018>#<Reference:0x0000555675d45e60>#<Reference:0x0000555675d45be0>#<Reference:0x0000555675d45960>#<Reference:0x0000555675d45780>#<Reference:0x0000555675d45500>#<Reference:0x0000555675d45320>#<Reference:0x0000555675d45140>#<Reference:0x0000555675d44ee8>#<Reference:0x0000555675d44ce0>#<Reference:0x0000555675d44ad8>#<Reference:0x0000555675d44858>#<Reference:0x0000555675d445b0>#<Reference:0x0000555675d443a8>Cytochrome P450, family 21, subfamily A, polypeptide 2Q08AG9CYP21A2