1.02011-09-21 00:05:37 UTC2015-07-21 06:56:54 UTCFDB0219442-Hydroxy-3-methylpentanoic acid2-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 acid2-Hydroxy-3-methyl-pentanoate2-Hydroxy-3-methyl-pentanoic acid2-hydroxy-3-methyl-Valerate2-hydroxy-3-methyl-Valeric acid2-Hydroxy-3-methylpentanoate2-Hydroxy-3-methylpentanoic acid2-Hydroxy-3-methylvalerate2-Hydroxy-3-methylvaleric acidA-hydroxy-b-methylvalerateA-hydroxy-b-methylvaleric acidalpha-hydroxy-beta-methylvaleratealpha-hydroxy-beta-methylvaleric acidHMVAC6H12O3132.1577132.07864425(2R,3R)-2-hydroxy-3-methylpentanoic acid2-hydroxy-3-methyl-valerate488-15-3CC[C@@H](C)[C@@H](O)C(O)=OInChI=1S/C6H12O3/c1-3-4(2)5(7)6(8)9/h4-5,7H,3H2,1-2H3,(H,8,9)/t4-,5-/m1/s1RILPIWOPNGRASR-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 acidsOrganic compoundsLipids and lipid-like moleculesFatty AcylsFatty acids and conjugatesAliphatic acyclic compoundsAlpha hydroxy acids and derivativesCarbonyl compoundsCarboxylic acidsHydrocarbon derivativesMethyl-branched fatty acidsMonocarboxylic acids and derivativesMonosaccharidesOrganic oxidesSecondary alcoholsAlcoholAliphatic acyclic compoundAlpha-hydroxy acidBranched fatty acidCarbonyl groupCarboxylic acidCarboxylic acid derivativeHydrocarbon derivativeHydroxy acidHydroxy fatty acidMethyl-branched fatty acidMonocarboxylic acid or derivativesMonosaccharideOrganic oxideOrganic oxygen compoundOrganooxygen compoundSecondary alcoholHydroxy fatty acidsSolidlogp0.74logs0.05solubility1.49e+02 g/llogp0.86pka_strongest_acidic4.25pka_strongest_basic-3.9iupac(2R,3R)-2-hydroxy-3-methylpentanoic acidaverage_mass132.1577mono_mass132.07864425smilesCC[C@@H](C)[C@@H](O)C(O)=OformulaC6H12O3inchiInChI=1S/C6H12O3/c1-3-4(2)5(7)6(8)9/h4-5,7H,3H2,1-2H3,(H,8,9)/t4-,5-/m1/s1inchikeyRILPIWOPNGRASR-RFZPGFLSSA-Npolar_surface_area57.53refractivity32.44polarizability13.77rotatable_bond_count3acceptor_count3donor_count2physiological_charge-1formal_charge0Specdb::MsIr404Specdb::MsIr405Specdb::MsIr406Specdb::NmrOneD340248Specdb::NmrOneD340249Specdb::NmrOneD340250Specdb::NmrOneD340251Specdb::NmrOneD340252Specdb::NmrOneD340253Specdb::NmrOneD340254Specdb::NmrOneD340255Specdb::NmrOneD340256Specdb::NmrOneD340257Specdb::NmrOneD340258Specdb::NmrOneD340259Specdb::NmrOneD340260Specdb::NmrOneD340261Specdb::NmrOneD340262Specdb::NmrOneD340263Specdb::NmrOneD340264Specdb::NmrOneD340265Specdb::NmrOneD340266Specdb::NmrOneD340267Specdb::CMs2976Specdb::CMs37425Specdb::CMs166941Specdb::CMs1058130Specdb::CMs1058132Specdb::CMs1058134Specdb::CMs1058135Specdb::CMs1058137Specdb::MsMs28841Specdb::MsMs28842Specdb::MsMs28843Specdb::MsMs35399Specdb::MsMs35400Specdb::MsMs35401Specdb::MsMs2244382Specdb::MsMs2245709Specdb::MsMs2246546Specdb::MsMs2816995Specdb::MsMs2816996Specdb::MsMs2816997Specdb::MsMs2888338Specdb::MsMs2888339Specdb::MsMs2888340HMDB00317#<Reference:0x0000555675a8f360>AnatidaeType 1specificAnatidae8830BeefaloType 1specificBos taurus X Bison bison297284BisonType 1specificBison bison9901BuffaloType 1specificBubalus bubalis89462Cattle (Beef, Veal)Type 1specificBos taurus9913ChickenType 1specificGallus gallus9031Columbidae (Dove, Pigeon)Type 1specificColumbidae8930DeerType 1specificCervidae9850Domestic goatType 1specificCapra aegagrus hircus9925Domestic pigType 1specificSus scrofa domestica9825ElkType 1specificCervus canadensis1574408EmuType 1specificDromaius novaehollandiae8790European rabbitType 1specificOryctolagus9984Greylag gooseType 1specificAnser anser8843Guinea henType 1specificNumida meleagris8996HorseType 1specificEquus caballus9796Mallard duckType 1specificAnas platyrhynchos8839Mountain hareType 1specificLepus timidus62621Mule deerType 1specificOdocoileus9871OstrichType 1specificStruthio camelus8801PheasantType 1specificPhasianus colchicus9054QuailType 1specificPhasianidae9005RabbitType 1specificLeporidae9979Rock ptarmiganType 1specificLagopus muta64668Sheep (Mutton, Lamb)Type 1specificOvis aries9940SquabType 1specificColumba8931TurkeyType 1specificMeleagris gallopavo9103Velvet duckType 1specificMelanitta fusca371864Wild boarType 1specificSus scrofa9823