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ACAA1

Domain

The PTS2-type peroxisomal targeting signal, which mediates interaction with PEX7 and localization to peroxisomes, is cleaved following import into peroxisomes.

Function

Responsible for the thiolytic cleavage of straight chain 3-keto fatty acyl-CoAs (3-oxoacyl-CoAs) (PubMed:11734571, PubMed:2882519). Plays an important role in fatty acid peroxisomal beta-oxidation (PubMed:11734571, PubMed:2882519). Catalyzes the cleavage of short, medium, long, and very long straight chain 3-oxoacyl-CoAs (PubMed:11734571, PubMed:2882519).

Pathway

Lipid metabolism; peroxisomal fatty acid beta-oxidation.

Sequence Similarities

Belongs to the thiolase-like superfamily. Thiolase family.

Cellular localization

Alternative names

ACAA, PTHIO, ACAA1, Acetyl-CoA C-myristoyltransferase, Acetyl-CoA acyltransferase, Beta-ketothiolase, Peroxisomal 3-oxoacyl-CoA thiolase

swissprot:P09110 omim:604054 entrezGene:30