1.0 2011-09-21 00:08:44 UTC 2019-11-26 03:21:01 UTC FDB022117 L-Alloisoleucine L-alloisoleucine is a branched chain amino acid and is a stereo-isomer of L-isoleucine. It is a common constituent of human plasma (albeit at low levels). L-alloisoleucine is produced as a byproduct of isoleucine transamination. L-Isoleucine is one of the four common amino acids (the 3 others being threonine, hydroxyproline, and hydroxylysine) that have two asymmetric carbon atoms which produce four structural possibilities for the same chemical composition. L-alloisoleucine differs from L-isoleucine by having having different stereochemistry around its beta carbon. Plasma L-alloisoleucine, which is derived from L-isoleucine in vivo, can be used for the diagnosis of maple syrup urine disease (MSUD). Indeed, plasma L-alloisoleucine levels above 5 umol/L is the most specific and most sensitive diagnostic marker for all forms of MSUD (PMID: 10508118). In MSUD the degradation of the essential branched-chain L-amino acids leucine, valine, and isoleucine and their derived 2-oxoacids is impaired because of an inherited deficiency in branched-chain 2-oxoacid dehydrogenase complex (EC 1.2.4.4) activity. The accumulation of branched-chain compounds in blood and other body fluids can exert neurotoxic effects. [HMDB] (2S,3R)-2-amino-3-methylpentanoate (2S,3R)-2-amino-3-methylpentanoic acid (3R)-LS-Isoleucine [S-(R*,S*)]-2-Amino-3-methylpentanoate [S-(R*,S*)]-2-Amino-3-methylpentanoic acid 2-amino-3-methyl-[S-(R*,S*)]-Pentanoate 2-amino-3-methyl-[S-(R*,S*)]-Pentanoic acid Alle Allo-Isoleucine Allo-L-Isoleucine L-allo-Isoleucine L-alloisoleucine L(+)-Alloisoleucine threo-3-methyl-L-Norvaline threo-L-Isoleucine C6H13NO2 131.1729 131.094628665 (2S,3R)-2-amino-3-methylpentanoic acid allo-isoleucine 1509-34-8 CC[C@@H](C)[C@H](N)C(O)=O InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5+/m1/s1 AGPKZVBTJJNPAG-UHNVWZDZSA-N belongs to the class of organic compounds known as isoleucine and derivatives. Isoleucine and derivatives are compounds containing isoleucine or a derivative thereof resulting from reaction of isoleucine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom. Isoleucine and derivatives Organic compounds Organic acids and derivatives Carboxylic acids and derivatives Amino acids, peptides, and analogues Aliphatic acyclic compounds Amino acids Carbonyl compounds Carboxylic acids Hydrocarbon derivatives L-alpha-amino acids Methyl-branched fatty acids Monoalkylamines Monocarboxylic acids and derivatives Organic oxides Organopnictogen compounds Aliphatic acyclic compound Alpha-amino acid Amine Amino acid Branched fatty acid Carbonyl group Carboxylic acid Fatty acid Fatty acyl Hydrocarbon derivative Isoleucine or derivatives L-alpha-amino acid Methyl-branched fatty acid Monocarboxylic acid or derivatives Organic nitrogen compound Organic oxide Organic oxygen compound Organonitrogen compound Organooxygen compound Organopnictogen compound Primary aliphatic amine Primary amine alloisoleucine non-proteinogenic L-alpha-amino acid Solid logp -1.73 ALOGPS logs -0.06 ALOGPS solubility 1.14e+02 g/l ALOGPS logp -1.5 ChemAxon pka_strongest_acidic 2.79 ChemAxon pka_strongest_basic 9.59 ChemAxon iupac (2S,3R)-2-amino-3-methylpentanoic acid ChemAxon average_mass 131.1729 ChemAxon mono_mass 131.094628665 ChemAxon smiles CC[C@@H](C)[C@H](N)C(O)=O ChemAxon formula C6H13NO2 ChemAxon inchi InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5+/m1/s1 ChemAxon inchikey AGPKZVBTJJNPAG-UHNVWZDZSA-N ChemAxon polar_surface_area 63.32 ChemAxon refractivity 34.09 ChemAxon polarizability 14.23 ChemAxon rotatable_bond_count 3 ChemAxon acceptor_count 3 ChemAxon donor_count 2 ChemAxon physiological_charge 0 ChemAxon formal_charge 0 ChemAxon Specdb::MsIr 631 Specdb::MsIr 632 Specdb::MsIr 633 Specdb::NmrTwoD 1363 Specdb::CMs 8795 Specdb::CMs 37621 Specdb::CMs 137461 Specdb::CMs 145195 Specdb::CMs 1065778 Specdb::CMs 1065780 Specdb::CMs 1065781 Specdb::NmrOneD 1419 Specdb::NmrOneD 144310 Specdb::NmrOneD 144311 Specdb::NmrOneD 144312 Specdb::NmrOneD 144313 Specdb::NmrOneD 144314 Specdb::NmrOneD 144315 Specdb::NmrOneD 144316 Specdb::NmrOneD 144317 Specdb::NmrOneD 144318 Specdb::NmrOneD 144319 Specdb::NmrOneD 144320 Specdb::NmrOneD 144321 Specdb::NmrOneD 144322 Specdb::NmrOneD 144323 Specdb::NmrOneD 144324 Specdb::NmrOneD 144325 Specdb::NmrOneD 144326 Specdb::NmrOneD 144327 Specdb::NmrOneD 144328 Specdb::NmrOneD 144329 Specdb::MsMs 779 Specdb::MsMs 780 Specdb::MsMs 781 Specdb::MsMs 261861 Specdb::MsMs 261862 Specdb::MsMs 261863 Specdb::MsMs 281793 Specdb::MsMs 281794 Specdb::MsMs 281795 Specdb::MsMs 2235673 Specdb::MsMs 2237706 Specdb::MsMs 2237747 Specdb::MsMs 2239809 Specdb::MsMs 2239905 Specdb::MsMs 2241820 Specdb::MsMs 2241991 Specdb::MsMs 2243862 Specdb::MsMs 2244065 Specdb::MsMs 2323027 Specdb::MsMs 2323028 Specdb::MsMs 2323029 Specdb::MsMs 2616342 Specdb::MsMs 2616343 Specdb::MsMs 2616344 HMDB00557 22359 IIL #<Reference:0x000055ce331c9790> #<Reference:0x000055ce331c95d8> 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 Milk (Cow) Type 2 specific 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