LMPD Database

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LMP001474

UniProt Annotations

Entry Information
Gene NameAU RNA binding protein/enoyl-CoA hydratase
Protein EntryAUHM_HUMAN
UniProt IDQ13825
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q13825-1; Sequence=Displayed; Name=2; IsoId=Q13825-2; Sequence=VSP_008336; Note=No experimental confirmation available.;
Catalytic Activity(S)-3-hydroxy-3-methylglutaryl-CoA = trans-3- methylglutaconyl-CoA + H(2)O.
Disease3-methylglutaconic aciduria 1 (MGA1) [MIM
FunctionCatalyzes the conversion of 3-methylglutaconyl-CoA to 3- hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs. {ECO
PathwayAmino-acid degradation; L-leucine degradation; (S)-3- hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA
SimilarityBelongs to the enoyl-CoA hydratase/isomerase family.
Subcellular LocationMitochondrion .
SubunitHomohexamer.