ACAA1

Acetyl-CoA acyltransferase 1
PDB rendering based on 2iik.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 2IIK
Identifiers
Symbols	ACAA1 ; ACAA; PTHIO; THIO
External IDs	OMIM: 604054 MGI: 2148491 HomoloGene: 37497 GeneCards: ACAA1 Gene
EC number	2.3.1.16
Gene ontology Molecular function • acetyl-CoA C-acyltransferase activity • protein binding • palmitoyl-CoA oxidase activity Cellular component • peroxisome • peroxisomal matrix • membrane • intracellular membrane-bounded organelle Biological process • very long-chain fatty acid metabolic process • fatty acid beta-oxidation • bile acid metabolic process • fatty acid beta-oxidation using acyl-CoA oxidase • unsaturated fatty acid metabolic process • alpha-linolenic acid metabolic process • cellular lipid metabolic process • small molecule metabolic process Sources: Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	30	113868
Ensembl	ENSG00000060971	ENSMUSG00000036138
UniProt	P09110	Q921H8
RefSeq (mRNA)	NM_001130410	NM_130864
RefSeq (protein)	NP_001123882	NP_570934
Location (UCSC)	Chr 3: 38.1 – 38.14 Mb	Chr 9: 119.34 – 119.35 Mb
PubMed search	[1]	[2]

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.

Further reading

This article incorporates text from the United States National Library of Medicine, which is in the public domain.