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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 5:116,294,409...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
            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 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
                                                  2-hydroxy-4-methylvaleric acid 1
                                                    (R)-2-hydroxy-4-methylpentanoic acid + 0
                                                    (S)-2-hydroxy-4-methylpentanoic acid 0
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