1.0 2011-09-21 00:10:31 UTC 2025-11-19 02:41:10 UTC FDB022225 3-Hydroxyisovaleric acid 3-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-Butanoate 3-hydroxy-3-methyl-Butanoic acid 3-hydroxy-3-methyl-Butyric acid 3-Hydroxy-3-methylbutanoate 3-Hydroxy-3-methylbutanoic acid 3-Hydroxy-3-methylbutyrate 3-Hydroxy-3-methylbutyric acid 3-Hydroxy-isovalerate 3-hydroxy-isovaleric acid 3-Hydroxyisovalerate 3-hydroxyisovaleric acid 3-OH-Isovalerate 3-OH-isovaleric acid B-hydroxy-b-methylbutyrate B-hydroxy-b-methylbutyric acid B-hydroxyisovalerate B-hydroxyisovaleric acid beta-hydroxy-beta-methylbutyrate beta-hydroxy-beta-methylbutyric acid beta-hydroxyisovalerate beta-hydroxyisovaleric acid HMB Hmb-D6 β-hydroxy-β-methylbutyrate β-hydroxy-β-methylbutyric acid β-hydroxyisovalerate β-hydroxyisovaleric acid C5H10O3 118.1311 118.062994186 3-hydroxy-3-methylbutanoic acid 3-hydroxyisovaleric acid 625-08-1 CC(C)(O)CC(O)=O InChI=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 acids Organic compounds Lipids and lipid-like molecules Fatty Acyls Fatty acids and conjugates Aliphatic acyclic compounds Carbonyl compounds Carboxylic acids Hydrocarbon derivatives Methyl-branched fatty acids Monocarboxylic acids and derivatives Organic oxides Short-chain hydroxy acids and derivatives Tertiary alcohols Alcohol Aliphatic acyclic compound Branched fatty acid Carbonyl group Carboxylic acid Carboxylic acid derivative Hydrocarbon derivative Hydroxy fatty acid Methyl-branched fatty acid Monocarboxylic acid or derivatives Organic oxide Organic oxygen compound Organooxygen compound Short-chain hydroxy acid Tertiary alcohol 3-hydroxy monocarboxylic acid Hydroxy fatty acids Solid logp -0.12 ALOGPS logs 0.51 ALOGPS solubility 3.84e+02 g/l ALOGPS logp -0.11 ChemAxon pka_strongest_acidic 4.55 ChemAxon pka_strongest_basic -2.7 ChemAxon iupac 3-hydroxy-3-methylbutanoic acid ChemAxon average_mass 118.1311 ChemAxon mono_mass 118.062994186 ChemAxon smiles CC(C)(O)CC(O)=O ChemAxon formula C5H10O3 ChemAxon inchi InChI=1S/C5H10O3/c1-5(2,8)3-4(6)7/h8H,3H2,1-2H3,(H,6,7) ChemAxon inchikey AXFYFNCPONWUHW-UHFFFAOYSA-N ChemAxon polar_surface_area 57.53 ChemAxon refractivity 28.1 ChemAxon polarizability 11.75 ChemAxon rotatable_bond_count 2 ChemAxon acceptor_count 3 ChemAxon donor_count 2 ChemAxon physiological_charge -1 ChemAxon formal_charge 0 ChemAxon Specdb::MsIr 831 Specdb::MsIr 832 Specdb::MsIr 833 Specdb::NmrTwoD 1469 Specdb::EiMs 748 Specdb::CMs 642 Specdb::CMs 24244 Specdb::CMs 30443 Specdb::CMs 37745 Specdb::CMs 146148 Specdb::CMs 1074721 Specdb::CMs 1074722 Specdb::CMs 1074724 Specdb::CMs 1074726 Specdb::CMs 1074727 Specdb::NmrOneD 1523 Specdb::NmrOneD 20762 Specdb::NmrOneD 20763 Specdb::NmrOneD 20764 Specdb::NmrOneD 20765 Specdb::NmrOneD 20766 Specdb::NmrOneD 20767 Specdb::NmrOneD 20768 Specdb::NmrOneD 20769 Specdb::NmrOneD 20770 Specdb::NmrOneD 20771 Specdb::NmrOneD 20772 Specdb::NmrOneD 20773 Specdb::NmrOneD 20774 Specdb::NmrOneD 20775 Specdb::NmrOneD 20776 Specdb::NmrOneD 20777 Specdb::NmrOneD 20778 Specdb::NmrOneD 20779 Specdb::NmrOneD 20780 Specdb::NmrOneD 20781 Specdb::NmrOneD 166332 Specdb::NmrOneD 166676 Specdb::MsMs 1080 Specdb::MsMs 1081 Specdb::MsMs 1082 Specdb::MsMs 319690 Specdb::MsMs 319691 Specdb::MsMs 319692 Specdb::MsMs 366889 Specdb::MsMs 366890 Specdb::MsMs 366891 Specdb::MsMs 2240054 Specdb::MsMs 2241645 Specdb::MsMs 2242101 Specdb::MsMs 2243787 Specdb::MsMs 2344292 Specdb::MsMs 2344293 Specdb::MsMs 2344294 Specdb::MsMs 2590939 Specdb::MsMs 2590940 Specdb::MsMs 2590941 HMDB00754 37084 #<Reference:0x000055ce332f2770> #<Reference:0x000055ce332f25b8> #<Reference:0x000055ce332f2400> #<Reference:0x000055ce332f2248> #<Reference:0x000055ce332f2090> #<Reference:0x000055ce332f1ed8> #<Reference:0x000055ce332f1d20> #<Reference:0x000055ce332f1b68> #<Reference:0x000055ce332f19b0> #<Reference:0x000055ce332f17f8> #<Reference:0x000055ce332f1640> #<Reference:0x000055ce332f1488>