1.02011-09-21 00:10:31 UTC2015-10-09 22:30:48 UTCFDB0222253-Hydroxyisovaleric acid3-Hydroxyisovaleric acid is a normal human metabolite excreted in the urine. Elevated levels of this compound are found in several inherited disorders such as Dihydrolipoamide dehydrogenase Deficiency, 3-Methylcrotonyl-CoA carboxylase 1 deficiency, 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (3-hydroxy-3-methylglutaryl -CoA lyase Deficiency, Biotinidase deficiency multiple carboxylase deficiency late-onset , Late onset multiple carboxylase deficiency, HolMcarboxylase synthetase deficiency, 3-Methylcrotonyl-CoA carboxylase 2 deficiency. 3-Hydroxyisovaleric acid is also elevated in smokers, in subjects undergoing long-term anticonvulsant therapy with carbamazepine and/or phenytoin. These levels are elevated due to impairment of renal reclamation of biotin. Levels may also be increased from prolonged consumption of raw egg-whites (PMID: 16895887, 9523856, 15447901, 9176832)(OMIM: 210210, 253270, 600529, 253260, 246450, 210200, 238331) [HMDB]3-hydroxy-3-methyl-Butanoate3-hydroxy-3-methyl-Butanoic acid3-hydroxy-3-methyl-Butyric acid3-Hydroxy-3-methylbutanoate3-Hydroxy-3-methylbutanoic acid3-Hydroxy-3-methylbutyrate3-Hydroxy-3-methylbutyric acid3-Hydroxy-isovalerate3-hydroxy-isovaleric acid3-Hydroxyisovalerate3-hydroxyisovaleric acid3-OH-Isovalerate3-OH-isovaleric acidB-hydroxy-b-methylbutyrateB-hydroxy-b-methylbutyric acidB-hydroxyisovalerateB-hydroxyisovaleric acidbeta-hydroxy-beta-methylbutyratebeta-hydroxy-beta-methylbutyric acidbeta-hydroxyisovaleratebeta-hydroxyisovaleric acidHMBHmb-D6β-hydroxy-β-methylbutyrateβ-hydroxy-β-methylbutyric acidβ-hydroxyisovalerateβ-hydroxyisovaleric acidC5H10O3118.1311118.0629941863-hydroxy-3-methylbutanoic acid3-hydroxyisovaleric acid625-08-1CC(C)(O)CC(O)=OInChI=1S/C5H10O3/c1-5(2,8)3-4(6)7/h8H,3H2,1-2H3,(H,6,7)AXFYFNCPONWUHW-UHFFFAOYSA-N belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.Hydroxy fatty acidsOrganic compoundsLipids and lipid-like moleculesFatty AcylsFatty acids and conjugatesAliphatic acyclic compoundsCarbonyl compoundsCarboxylic acidsHydrocarbon derivativesMethyl-branched fatty acidsMonocarboxylic acids and derivativesOrganic oxidesShort-chain hydroxy acids and derivativesTertiary alcoholsAlcoholAliphatic acyclic compoundBranched fatty acidCarbonyl groupCarboxylic acidCarboxylic acid derivativeHydrocarbon derivativeHydroxy fatty acidMethyl-branched fatty acidMonocarboxylic acid or derivativesOrganic oxideOrganic oxygen compoundOrganooxygen compoundShort-chain hydroxy acidTertiary alcohol3-hydroxy monocarboxylic acidHydroxy fatty acidsSolidlogp-0.12logs0.51solubility3.84e+02 g/llogp-0.11pka_strongest_acidic4.55pka_strongest_basic-2.7iupac3-hydroxy-3-methylbutanoic acidaverage_mass118.1311mono_mass118.062994186smilesCC(C)(O)CC(O)=OformulaC5H10O3inchiInChI=1S/C5H10O3/c1-5(2,8)3-4(6)7/h8H,3H2,1-2H3,(H,6,7)inchikeyAXFYFNCPONWUHW-UHFFFAOYSA-Npolar_surface_area57.53refractivity28.1polarizability11.75rotatable_bond_count2acceptor_count3donor_count2physiological_charge-1formal_charge0Specdb::EiMs748Specdb::NmrTwoD1469Specdb::MsIr831Specdb::MsIr832Specdb::MsIr833Specdb::CMs642Specdb::CMs24244Specdb::CMs30443Specdb::CMs37745Specdb::CMs146148Specdb::CMs1074721Specdb::CMs1074722Specdb::CMs1074724Specdb::CMs1074726Specdb::CMs1074727Specdb::NmrOneD1523Specdb::NmrOneD20762Specdb::NmrOneD20763Specdb::NmrOneD20764Specdb::NmrOneD20765Specdb::NmrOneD20766Specdb::NmrOneD20767Specdb::NmrOneD20768Specdb::NmrOneD20769Specdb::NmrOneD20770Specdb::NmrOneD20771Specdb::NmrOneD20772Specdb::NmrOneD20773Specdb::NmrOneD20774Specdb::NmrOneD20775Specdb::NmrOneD20776Specdb::NmrOneD20777Specdb::NmrOneD20778Specdb::NmrOneD20779Specdb::NmrOneD20780Specdb::NmrOneD20781Specdb::NmrOneD166332Specdb::NmrOneD166676Specdb::MsMs2241645Specdb::MsMs2242101Specdb::MsMs2243787Specdb::MsMs1080Specdb::MsMs1081Specdb::MsMs1082Specdb::MsMs2240054Specdb::MsMs366889Specdb::MsMs366890Specdb::MsMs366891Specdb::MsMs2590939Specdb::MsMs2590940Specdb::MsMs2590941Specdb::MsMs319690Specdb::MsMs319691Specdb::MsMs319692Specdb::MsMs2344292Specdb::MsMs2344293Specdb::MsMs2344294HMDB0075437084#<Reference:0x0000555675fd5ba8>#<Reference:0x0000555675fd59a0>#<Reference:0x0000555675fd57c0>#<Reference:0x0000555675fd55b8>#<Reference:0x0000555675fd5400>#<Reference:0x0000555675fd51f8>#<Reference:0x0000555675fd4fc8>#<Reference:0x0000555675fd4dc0>#<Reference:0x0000555675fd4b68>#<Reference:0x0000555675fd48e8>#<Reference:0x0000555675fd4690>#<Reference:0x0000555675fd4460>