LMPD Database

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LMP002274

UniProt Annotations

Entry Information
Gene Nameacyl-Coenzyme A oxidase 1, palmitoyl
Protein EntryACOX1_MOUSE
UniProt IDQ9R0H0
SpeciesMouse
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q9R0H0-1; Sequence=Displayed; Name=2; IsoId=Q9R0H0-2; Sequence=VSP_042477;
Catalytic ActivityAcyl-CoA + O(2) = trans-2,3-dehydroacyl-CoA + H(2)O(2).
CofactorName=FAD; Xref=ChEBI:CHEBI:57692; Evidence={ECO:0000250};
Disruption PhenotypeSevere microvesicular hepatic steatosis, sustained activation of Ppara, spontaneous massive peroxisome proliferation and eventual development of hepatocellular carcinomas. {ECO:0000269|PubMed:20195242}.
FunctionCatalyzes the desaturation of acyl-CoAs to 2-trans- enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs (By similarity). {ECO:0000250}.
PathwayLipid metabolism; peroxisomal fatty acid beta-oxidation.
Sequence CautionSequence=BAE41642.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence={ECO:0000305};
SimilarityBelongs to the acyl-CoA oxidase family. {ECO:0000305}.
Subcellular LocationPeroxisome {ECO:0000250}.
Tissue SpecificityHighest levels of isoform 1 are found in liver and kidney while highest levels of isoform 2 are found in white adipose tissue. Isoform 1 is expressed at higher levels than isoform 2 in liver and kidney while isoform 2 is expressed at higher levels in brain, heart, lung, muscle, white adipose tissue and testis. {ECO:0000269|PubMed:20195242}.