LMPD Database

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LMP002411

UniProt Annotations

Entry Information
Gene Nameacyl-CoA oxidase 1, palmitoyl
Protein EntryACOX1_HUMAN
UniProt IDQ15067
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; Synonyms=ACOX1a, SCOX-exon 3I; IsoId=Q15067-1; Sequence=Displayed; Name=2; Synonyms=ACOX1b, SCOX-exon 3II; IsoId=Q15067-2; Sequence=VSP_000146; Name=3; IsoId=Q15067-3; Sequence=VSP_046129, VSP_000146; Note=No experimental confirmation available.;
Biophysicochemical PropertiesKinetic parameters: KM=73 uM for palmitoyl-CoA (isoform 1) ; KM=90 uM for palmitoyl-CoA (isoform 2) ; pH dependence: Optimum pH is 8.5 for isoform 1 and 7.5-8.5 for isoform 2. ; Temperature dependence: Optimum temperature for isoform 1 at pH 7.5 is 40 degrees Celsius with no activity at 50 degrees Celsius. Optimum temperature for isoform 2 at pH 7.5 is 47.5 degrees Celsius with 57% activity retained at 50 degrees Celsius. ;
Catalytic ActivityAcyl-CoA + O(2) = trans-2,3-dehydroacyl-CoA + H(2)O(2).
CofactorName=FAD; Xref=ChEBI
DiseaseAdrenoleukodystrophy, pseudoneonatal (Pseudo-NALD) [MIM
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. Isoform 2 is twice as active as isoform 1 against 16-hydroxy- palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl- CoA.
MiscellaneousIsoform 1 and isoform 2 can reverse the Acox1 null phenotype in mouse which is characterized by severe microvesicular hepatic steatosis, sustained activation of Ppara, spontaneous massive peroxisome proliferation and eventual development of hepatocellular carcinomas. Isoform 2 is more effective in reversal of the phenotype than isoform 1 (PubMed:20195242).
PathwayLipid metabolism; peroxisomal fatty acid beta-oxidation.
Sequence CautionSequence=CAD97622.1; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence= ;
SimilarityBelongs to the acyl-CoA oxidase family.
Subcellular LocationPeroxisome.
Tissue SpecificityWidely expressed with highest levels of isoform 1 and isoform 2 detected in testis. Isoform 1 is expressed at higher levels than isoform 2 in liver and kidney while isoform 2 levels are higher in brain, lung, muscle, white adipose tissue and testis. Levels are almost equal in heart.