title: ACAA1
text: # ACAA1
  
  3-Ketoacyl-CoA thiolase, peroxisomal also known as acetyl-Coenzyme A acyltransferase 1 is an enzyme that in humans is encoded by the ACAA1 gene.
  
  Acetyl-Coenzyme A acyltransferase 1 is an acetyl-CoA C-acyltransferase enzyme.
  
  ## Function
  
  This gene encodes an enzyme operative in the beta oxidation system of the peroxisomes.
  
  ## Clinical significance
  
  Deficiency of this enzyme leads to pseudo-Zellweger syndrome.
  
  