1.0 2011-09-21 00:05:37 UTC 2015-07-21 06:56:54 UTC FDB021944 2-Hydroxy-3-methylpentanoic acid 2-Hydroxy-3-methylpentanoic acid or 2-hydroxy-3-methylvaleric acid (HMVA) is an organic acid generated by L-isoleucine metabolism. It is derived from the reduction of 2-Keto-3-methylvaleric acid (KMVA), possibly through the action of a lactate dehydrogenase (PMID: 1429566). There are 4 stereoisomers of HMVA (2S,3S-HMVA, 2R,3R-HMVA, 2S,3R-HMVA and 2R,3S-HMVA), of which the 2S,3S and 2S,3R derivatives are generally separable. HMVA is found in the urine and blood of normal individuals but in very elevated levels in patients with maple syrup urine disease (MSUD) (PMID: 1429566). Maple syrup urine disease (MSUD) is an inherited metabolic disease predominantly characterized by neurological dysfunction including psychomotor/delay/mental retardation. [HMDB] (2R,3R)-2-hydroxy-3-methyl-pentanoate (2R,3R)-2-hydroxy-3-methyl-pentanoic acid (2R,3R)-2-hydroxy-3-methylpentanoate (2R,3R)-2-hydroxy-3-methylpentanoic acid 2-Hydroxy-3-methyl-pentanoate 2-Hydroxy-3-methyl-pentanoic acid 2-hydroxy-3-methyl-Valerate 2-hydroxy-3-methyl-Valeric acid 2-Hydroxy-3-methylpentanoate 2-Hydroxy-3-methylpentanoic acid 2-Hydroxy-3-methylvalerate 2-Hydroxy-3-methylvaleric acid A-hydroxy-b-methylvalerate A-hydroxy-b-methylvaleric acid alpha-hydroxy-beta-methylvalerate alpha-hydroxy-beta-methylvaleric acid HMVA C6H12O3 132.1577 132.07864425 (2R,3R)-2-hydroxy-3-methylpentanoic acid 2-hydroxy-3-methyl-valerate 488-15-3 CC[C@@H](C)[C@@H](O)C(O)=O InChI=1S/C6H12O3/c1-3-4(2)5(7)6(8)9/h4-5,7H,3H2,1-2H3,(H,8,9)/t4-,5-/m1/s1 RILPIWOPNGRASR-RFZPGFLSSA-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 Alpha hydroxy acids and derivatives Carbonyl compounds Carboxylic acids Hydrocarbon derivatives Methyl-branched fatty acids Monocarboxylic acids and derivatives Monosaccharides Organic oxides Secondary alcohols Alcohol Aliphatic acyclic compound Alpha-hydroxy acid Branched fatty acid Carbonyl group Carboxylic acid Carboxylic acid derivative Hydrocarbon derivative Hydroxy acid Hydroxy fatty acid Methyl-branched fatty acid Monocarboxylic acid or derivatives Monosaccharide Organic oxide Organic oxygen compound Organooxygen compound Secondary alcohol Hydroxy fatty acids Solid logp 0.74 ALOGPS logs 0.05 ALOGPS solubility 1.49e+02 g/l ALOGPS logp 0.86 ChemAxon pka_strongest_acidic 4.25 ChemAxon pka_strongest_basic -3.9 ChemAxon iupac (2R,3R)-2-hydroxy-3-methylpentanoic acid ChemAxon average_mass 132.1577 ChemAxon mono_mass 132.07864425 ChemAxon smiles CC[C@@H](C)[C@@H](O)C(O)=O ChemAxon formula C6H12O3 ChemAxon inchi InChI=1S/C6H12O3/c1-3-4(2)5(7)6(8)9/h4-5,7H,3H2,1-2H3,(H,8,9)/t4-,5-/m1/s1 ChemAxon inchikey RILPIWOPNGRASR-RFZPGFLSSA-N ChemAxon polar_surface_area 57.53 ChemAxon refractivity 32.44 ChemAxon polarizability 13.77 ChemAxon rotatable_bond_count 3 ChemAxon acceptor_count 3 ChemAxon donor_count 2 ChemAxon physiological_charge -1 ChemAxon formal_charge 0 ChemAxon Specdb::MsIr 404 Specdb::MsIr 405 Specdb::MsIr 406 Specdb::CMs 2976 Specdb::CMs 37425 Specdb::CMs 166941 Specdb::CMs 1058130 Specdb::CMs 1058132 Specdb::CMs 1058134 Specdb::CMs 1058135 Specdb::CMs 1058137 Specdb::NmrOneD 340248 Specdb::NmrOneD 340249 Specdb::NmrOneD 340250 Specdb::NmrOneD 340251 Specdb::NmrOneD 340252 Specdb::NmrOneD 340253 Specdb::NmrOneD 340254 Specdb::NmrOneD 340255 Specdb::NmrOneD 340256 Specdb::NmrOneD 340257 Specdb::NmrOneD 340258 Specdb::NmrOneD 340259 Specdb::NmrOneD 340260 Specdb::NmrOneD 340261 Specdb::NmrOneD 340262 Specdb::NmrOneD 340263 Specdb::NmrOneD 340264 Specdb::NmrOneD 340265 Specdb::NmrOneD 340266 Specdb::NmrOneD 340267 Specdb::MsMs 28841 Specdb::MsMs 28842 Specdb::MsMs 28843 Specdb::MsMs 35399 Specdb::MsMs 35400 Specdb::MsMs 35401 Specdb::MsMs 2244382 Specdb::MsMs 2245709 Specdb::MsMs 2246546 Specdb::MsMs 2816995 Specdb::MsMs 2816996 Specdb::MsMs 2816997 Specdb::MsMs 2888338 Specdb::MsMs 2888339 Specdb::MsMs 2888340 HMDB00317 #<Reference:0x000055ce2ed7ed88> Anatidae Type 1 specific Anatidae 8830 Beefalo Type 1 specific Bos taurus X Bison bison 297284 Bison Type 1 specific Bison bison 9901 Buffalo Type 1 specific Bubalus bubalis 89462 Cattle (Beef, Veal) Type 1 specific Bos taurus 9913 Chicken Type 1 specific Gallus gallus 9031 Columbidae (Dove, Pigeon) Type 1 specific Columbidae 8930 Deer Type 1 specific Cervidae 9850 Domestic goat Type 1 specific Capra aegagrus hircus 9925 Domestic pig Type 1 specific Sus scrofa domestica 9825 Elk Type 1 specific Cervus canadensis 1574408 Emu Type 1 specific Dromaius novaehollandiae 8790 European rabbit Type 1 specific Oryctolagus 9984 Greylag goose Type 1 specific Anser anser 8843 Guinea hen Type 1 specific Numida meleagris 8996 Horse Type 1 specific Equus caballus 9796 Mallard duck Type 1 specific Anas platyrhynchos 8839 Mountain hare Type 1 specific Lepus timidus 62621 Mule deer Type 1 specific Odocoileus 9871 Ostrich Type 1 specific Struthio camelus 8801 Pheasant Type 1 specific Phasianus colchicus 9054 Quail Type 1 specific Phasianidae 9005 Rabbit Type 1 specific Leporidae 9979 Rock ptarmigan Type 1 specific Lagopus muta 64668 Sheep (Mutton, Lamb) Type 1 specific Ovis aries 9940 Squab Type 1 specific Columba 8931 Turkey Type 1 specific Meleagris gallopavo 9103 Velvet duck Type 1 specific Melanitta fusca 371864 Wild boar Type 1 specific Sus scrofa 9823