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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 5:116,294,409...116,477,904
Ensembl chr 5:116,289,823...116,475,908
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Term paths to the root
Path 1
Term Annotations click to browse term
  CHEBI ontology 19900
    role 19875
      biological role 19873
        biochemical role 19567
          metabolite 19552
            eukaryotic metabolite 19362
              plant metabolite 17927
                valeric acid 14535
                  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 19900
    subatomic particle 19898
      composite particle 19898
        hadron 19898
          baryon 19898
            nucleon 19898
              atomic nucleus 19898
                atom 19898
                  main group element atom 19845
                    p-block element atom 19845
                      carbon group element atom 19792
                        carbon atom 19787
                          organic molecular entity 19787
                            organic group 18957
                              organic divalent group 18938
                                organodiyl group 18938
                                  carbonyl group 18890
                                    carbonyl compound 18890
                                      carboxylic acid 18611
                                        monocarboxylic acid 17936
                                          fatty acid 16596
                                            saturated fatty acid 16279
                                              straight-chain saturated fatty acid 15745
                                                valeric acid 14535
                                                  3-methyl-2-oxovaleric acid 1
                                                    (R)-3-methyl-2-oxovaleric acid 0
                                                    (S)-3-methyl-2-oxovaleric acid 0
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