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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:2-hydroxy-4-methylvaleric acid
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Accession:CHEBI:59783 term browser browse the term
Definition:A valeric acid derivative having a hydroxy substituent at the 2-position and a methyl substituent at the 4-position; an alpha-hydroxy analogue of leucine. A bacterial metabolite, it has also been isolated from amniotic fluid, was found in a patient with dihydrolipoyl dehydrogenase deficiency and is present in the urine of patients with short bowel syndrome.
Synonyms:related_synonym: 2-Hydroxy-4-methylpentanoic acid;   2-hydroxyisocaproic acid;   2-hydroxyisohexanoic acid;   Formula=C6H12O3;   InChI=1S/C6H12O3/c1-4(2)3-5(7)6(8)9/h4-5,7H,3H2,1-2H3,(H,8,9);   InChIKey=LVRFTAZAXQPQHI-UHFFFAOYSA-N;   Leucic acid;   SMILES=C(C(C(O)=O)O)C(C)C;   alpha-Hydroxyisocaproic acid;   leucinic acid
 xref: CAS:498-36-2;   HMDB:HMDB0000665;   LIPID_MAPS_instance:LMFA01050381
 xref_mesh: MESH:C019419
 xref: PMID:17439666;   PMID:4018104;   PMID:6467607;   PMID:6688766;   Reaxys:1721733
 cyclic_relationship: is_conjugate_acid_of CHEBI:133577

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2-hydroxy-4-methylvaleric 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-hydroxyisocaproic 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 19785
    role 19732
      biological role 19732
        biochemical role 19281
          metabolite 19262
            2-hydroxy-4-methylvaleric acid 1
              (R)-2-hydroxy-4-methylpentanoic acid + 0
              (S)-2-hydroxy-4-methylpentanoic acid 0
Path 2
Term Annotations click to browse term
  CHEBI ontology 19785
    subatomic particle 19782
      composite particle 19782
        hadron 19782
          baryon 19782
            nucleon 19782
              atomic nucleus 19782
                atom 19782
                  main group element atom 19670
                    p-block element atom 19670
                      carbon group element atom 19572
                        carbon atom 19561
                          organic molecular entity 19561
                            organic group 18493
                              organic divalent group 18486
                                organodiyl group 18486
                                  carbonyl group 18389
                                    carbonyl compound 18389
                                      carboxylic acid 18060
                                        monocarboxylic acid 17406
                                          fatty acid 15844
                                            saturated fatty acid 15811
                                              straight-chain saturated fatty acid 15226
                                                valeric acid 14334
                                                  2-hydroxy-4-methylvaleric acid 1
                                                    (R)-2-hydroxy-4-methylpentanoic acid + 0
                                                    (S)-2-hydroxy-4-methylpentanoic acid 0
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