1.0 2011-09-21 00:26:20 UTC 2015-10-09 22:32:51 UTC FDB023208 beta-Leucine Beta-leucine is a metabolite that is in the middle of a controversy over its presence in the human body. While there are reports that claim it as a human metabolite, there are others that deny its existence. Two examples: Circulating levels of beta-leucine are elevated in the cobalamin-deficient state of pernicious anemia. Levels of leucine, on the other hand, are much lower. It is proposed that leucine 2,3-aminomutase, the cobalamin-dependent enzyme that catalyzes the interconversion of leucine and beta-leucine, is the affected enzyme in pernicious anemia and causes these results by preventing the synthesis of leucine from beta-leucine. The synthesis of leucine by human leukocytes and hair roots and by rat liver extracts has been shown to occur when either branched chain fatty acids or valine metabolites are the substances. The synthesis is dependent upon adenosylcobalamin and is inhibited by intrinsic factor. (PMID: 7430116) Using forms of beta-leucine and leucine that contain several deuterium atoms in place of several hydrogen atoms as internal standards, techniques have been developed which make it possible to detect and quantitate as little as 0.1 mumol/liter of beta-leucine or leucine in human serum and in incubations containing rat liver supernatant. beta-Leucine was not detectable, i.e. less than 0.1 mumol/liter, in any sera from 50 normal human subjects or in any sera from 50 cobalamin-deficient patients. Experiments in which beta-leucine, leucine, isostearic acid, or isocaproic acid were incubated with rat liver supernatant in the presence or absence of adenosylcobalamin or cobalamin-binding protein failed to demonstrate the formation of leucine or beta-leucine or their interconversion under any of the conditions studied. We conclude that beta-leucine is not present in human blood and that the existence of leucine 2,3-aminomutase in mammalian tissues remains to be established. (PMID 3356699) [HMDB] beta-2-Amino-4-methylvaleric acid beta-Leucine L-beta-Leucine C6H13NO2 131.1729 131.094628665 (3S)-3-amino-4-methylpentanoic acid β-leucine 5699-54-7 CC(C)[C@@H](N)CC(O)=O InChI=1S/C6H13NO2/c1-4(2)5(7)3-6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t5-/m0/s1 GLUJNGJDHCTUJY-YFKPBYRVSA-N belongs to the class of organic compounds known as beta amino acids and derivatives. These are amino acids having a (-NH2) group attached to the beta carbon atom. Beta amino acids and derivatives Organic compounds Organic acids and derivatives Carboxylic acids and derivatives Amino acids, peptides, and analogues Aliphatic acyclic compounds Amino acids Amino fatty acids Carbonyl compounds Carboxylic acids Hydrocarbon derivatives Methyl-branched fatty acids Monoalkylamines Monocarboxylic acids and derivatives Organic oxides Organopnictogen compounds Aliphatic acyclic compound Amine Amino acid Amino fatty acid Beta amino acid or derivatives Branched fatty acid Carbonyl group Carboxylic acid Fatty acid Fatty acyl Hydrocarbon derivative 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 beta-leucine Solid logp -2.14 ALOGPS logs -0.02 ALOGPS solubility 1.26e+02 g/l ALOGPS logp -1.9 ChemAxon pka_strongest_acidic 4.39 ChemAxon pka_strongest_basic 10.6 ChemAxon iupac (3S)-3-amino-4-methylpentanoic acid ChemAxon average_mass 131.1729 ChemAxon mono_mass 131.094628665 ChemAxon smiles CC(C)[C@@H](N)CC(O)=O ChemAxon formula C6H13NO2 ChemAxon inchi InChI=1S/C6H13NO2/c1-4(2)5(7)3-6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t5-/m0/s1 ChemAxon inchikey GLUJNGJDHCTUJY-YFKPBYRVSA-N ChemAxon polar_surface_area 63.32 ChemAxon refractivity 34.12 ChemAxon polarizability 14.07 ChemAxon rotatable_bond_count 3 ChemAxon acceptor_count 3 ChemAxon donor_count 2 ChemAxon physiological_charge 0 ChemAxon formal_charge 0 ChemAxon Specdb::NmrTwoD 1915 Specdb::MsIr 2028 Specdb::MsIr 2029 Specdb::MsIr 2030 Specdb::NmrOneD 1980 Specdb::NmrOneD 28482 Specdb::NmrOneD 28483 Specdb::NmrOneD 28484 Specdb::NmrOneD 28485 Specdb::NmrOneD 28486 Specdb::NmrOneD 28487 Specdb::NmrOneD 28488 Specdb::NmrOneD 28489 Specdb::NmrOneD 28490 Specdb::NmrOneD 28491 Specdb::NmrOneD 28492 Specdb::NmrOneD 28493 Specdb::NmrOneD 28494 Specdb::NmrOneD 28495 Specdb::NmrOneD 28496 Specdb::NmrOneD 28497 Specdb::NmrOneD 28498 Specdb::NmrOneD 28499 Specdb::NmrOneD 28500 Specdb::NmrOneD 28501 Specdb::CMs 13880 Specdb::CMs 38643 Specdb::CMs 131863 Specdb::CMs 139597 Specdb::MsMs 2337 Specdb::MsMs 2338 Specdb::MsMs 2339 Specdb::MsMs 5985 Specdb::MsMs 5986 Specdb::MsMs 5987 Specdb::MsMs 5988 Specdb::MsMs 5989 Specdb::MsMs 296617 Specdb::MsMs 296618 Specdb::MsMs 296619 Specdb::MsMs 337948 Specdb::MsMs 337949 Specdb::MsMs 337950 Specdb::MsMs 446450 Specdb::MsMs 446451 Specdb::MsMs 446452 Specdb::MsMs 446453 Specdb::MsMs 446454 Specdb::MsMs 2407499 Specdb::MsMs 2407500 Specdb::MsMs 2407501 Specdb::MsMs 2531163 Specdb::MsMs 2531164 Specdb::MsMs 2531165 HMDB03640 15604 #<Reference:0x000055ce30638a90> #<Reference:0x000055ce306388d8> 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