Anti-ACAA1

This gene encodes an enzyme operative in the beta-oxidation system of the peroxisomes. Deficiency of this enzyme leads to pseudo-Zellweger syndrome. Alternative splicing results in multiple transcript variants. Protein function: Responsible for the thiolytic cleavage of straight chain 3- keto fatty acyl-CoAs (3-oxoacyl-CoAs) (PubMed:2882519, PubMed:11734571). Plays an important role in fatty acid peroxisomal beta-oxidation (PubMed:2882519, PubMed:11734571). Catalyzes the cleavage of short, medium, long, and very long straight chain 3- oxoacyl-CoAs (PubMed:2882519, PubMed:11734571). [The UniProt Consortium]