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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 19821
    role 19769
      biological role 19769
        biochemical role 19389
          metabolite 19364
            eukaryotic metabolite 19052
              plant metabolite 17458
                valeric acid 14349
                  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 19821
    subatomic particle 19819
      composite particle 19819
        hadron 19819
          baryon 19819
            nucleon 19819
              atomic nucleus 19819
                atom 19819
                  main group element atom 19716
                    p-block element atom 19716
                      carbon group element atom 19640
                        carbon atom 19630
                          organic molecular entity 19630
                            organic group 18737
                              organic divalent group 18728
                                organodiyl group 18728
                                  carbonyl group 18675
                                    carbonyl compound 18675
                                      carboxylic acid 18373
                                        monocarboxylic acid 17636
                                          fatty acid 16008
                                            saturated fatty acid 15984
                                              straight-chain saturated fatty acid 15440
                                                valeric acid 14349
                                                  3-methyl-2-oxovaleric acid 1
                                                    (R)-3-methyl-2-oxovaleric acid 0
                                                    (S)-3-methyl-2-oxovaleric acid 0
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