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The Chemical Entities of Biological Interest (ChEBI) ontology is downloaded weekly from EMBL-EBI at The data is made available under the Creative Commons License (CC BY 3.0, For more information see: Degtyarenko et al. (2008) ChEBI: a database and ontology for chemical entities of biological interest. Nucleic Acids Res. 36, D344–D350.

Term:3-methyl-2-oxovaleric acid
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Accession:CHEBI:35932 term browser browse the term
Definition:A 2-oxo monocarboxylic acid that is valeric acid carrying oxo- and methyl substituents at C-2 and C-3, respectively. An alpha-keto acid analogue and metabolite of isoleucine in man, animals and bacteria. Used as a clinical marker for maple syrup urine disease (MSUD).
Synonyms:related_synonym: 2-Oxo-3-methyl-n-valeric acid;   2-Oxo-3-methylpentanoic acid;   2-Oxo-3-methylvalerate;   2-Oxo-3-methylvaleric acid;   2-Oxoisoleucine;   2-oxokolavenic acid;   3-Methyl-2-oxopentanoic acid;   3-ethyl-3-methylpyruvic acid;   Formula=C6H10O3;   InChI=1S/C6H10O3/c1-3-4(2)5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9);   InChIKey=JVQYSWDUAOAHFM-UHFFFAOYSA-N;   SMILES=CCC(C)C(=O)C(O)=O;   alpha-Oxo-beta-methylvaleric acid;   alpha-keto-beta-methyl-n-valeric acid;   alpha-keto-beta-methylvaleric acid;   alpha-oxo-beta-methyl-n-valeric acid;   ketoisoleucine
 xref: Beilstein:1098987;   CAS:1460-34-0;   Gmelin:971983;   HMDB:HMDB0000491;   KEGG:C03465
 xref_mesh: MESH:C016211
 xref: PMID:1638756;   PMID:17190852;   PMID:759179;   Reaxys:1098987
 cyclic_relationship: is_conjugate_acid_of CHEBI:28654

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3-methyl-2-oxovaleric acid term browser
Symbol Object Name Qualifiers Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G Lepr leptin receptor affects abundance ISO LEPR affects the abundance of alpha-keto-beta-methylvaleric acid CTD PMID:20567778 NCBI chr 5:120,503,475...120,682,281
Ensembl chr 5:120,564,645...120,682,221
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Term paths to the root
Path 1
Term Annotations click to browse term
  CHEBI ontology 19810
    role 19758
      biological role 19758
        biochemical role 19304
          metabolite 19285
            eukaryotic metabolite 18934
              plant metabolite 17422
                valeric acid 14339
                  3-methyl-2-oxovaleric acid 1
                    (R)-3-methyl-2-oxovaleric acid 0
                    (S)-3-methyl-2-oxovaleric acid 0
Path 2
Term Annotations click to browse term
  CHEBI ontology 19810
    subatomic particle 19808
      composite particle 19808
        hadron 19808
          baryon 19808
            nucleon 19808
              atomic nucleus 19808
                atom 19808
                  main group element atom 19696
                    p-block element atom 19696
                      carbon group element atom 19599
                        carbon atom 19588
                          organic molecular entity 19588
                            organic group 18527
                              organic divalent group 18520
                                organodiyl group 18520
                                  carbonyl group 18427
                                    carbonyl compound 18427
                                      carboxylic acid 18108
                                        monocarboxylic acid 17475
                                          fatty acid 15994
                                            saturated fatty acid 15967
                                              straight-chain saturated fatty acid 15418
                                                valeric acid 14339
                                                  3-methyl-2-oxovaleric acid 1
                                                    (R)-3-methyl-2-oxovaleric acid 0
                                                    (S)-3-methyl-2-oxovaleric acid 0
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