Send us a Message

Submit Data |  Help |  Video Tutorials |  News |  Publications |  Download |  REST API |  Citing RGD |  Contact   


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
go back to main search page
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

show annotations for term's descendants           Sort by:
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
JBrowse link

Term paths to the root
Path 1
Term Annotations click to browse term
  CHEBI ontology 20089
    role 20042
      biological role 20011
        biochemical role 19671
          metabolite 19631
            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 20089
    subatomic particle 20058
      composite particle 20058
        hadron 20088
          baryon 20088
            nucleon 20088
              atomic nucleus 20088
                atom 20058
                  main group element atom 19960
                    p-block element atom 19990
                      carbon group element atom 19916
                        carbon atom 19909
                          organic molecular entity 19909
                            organic group 18988
                              organic divalent group 18950
                                organodiyl group 18974
                                  carbonyl group 18903
                                    carbonyl compound 18925
                                      carboxylic acid 18597
                                        monocarboxylic acid 17843
                                          fatty acid 16480
                                            saturated fatty acid 16128
                                              straight-chain saturated fatty acid 15570
                                                valeric acid 14413
                                                  2-hydroxy-4-methylvaleric acid 1
                                                    (R)-2-hydroxy-4-methylpentanoic acid + 0
                                                    (S)-2-hydroxy-4-methylpentanoic acid 0
paths to the root