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MGP004804

UniProt Annotations

Entry Information
Gene Name2-hydroxyacyl-CoA lyase 1
Protein EntryHACL1_HUMAN
UniProt IDQ9UJ83
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=4; Name=1; IsoId=Q9UJ83-1; Sequence=Displayed; Name=2; IsoId=Q9UJ83-2; Sequence=VSP_054191; Note=No experimental confirmation available.; Name=4; IsoId=Q9UJ83-4; Sequence=VSP_054749, VSP_054750; Name=3; IsoId=Q9UJ83-3; Sequence=VSP_054192; Note=No experimental confirmation available.;
Catalytic Activity2-hydroxyphytanoyl-CoA = pristanal + formyl- CoA.
CofactorName=Mg(2+); Xref=ChEBI:CHEBI:18420; Note=Binds 1 Mg(2+) ion per subunit.;
CofactorName=thiamine diphosphate; Xref=ChEBI:CHEBI:58937; Note=Binds 1 thiamine pyrophosphate per subunit.;
FunctionCatalyzes a carbon-carbon cleavage reaction; cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA and a 2-methyl-branched fatty aldehyde.
InteractionSelf; NbExp=2; IntAct=EBI-746580, EBI-746580;
PathwayLipid metabolism; fatty acid metabolism.
Sequence CautionSequence=AAF28957.1; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence={ECO:0000305};
SimilarityBelongs to the TPP enzyme family. {ECO:0000305}.
Subcellular LocationPeroxisome.
SubunitHomotetramer.
Tissue SpecificityExpressed in high levels in liver, also expressed in kidney, heart and skeletal muscle. {ECO:0000269|PubMed:10468558}.
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