Acetyl Coenzyme A Acyltransferase 1 (ACAA1)

ACAA; PTHIO; Peroxisomal 3-Oxoacyl-Coenzyme A Thiolase; Acetyl-CoA acyltransferase; Beta-ketothiolase

SPN	T126
HGNC	82
MGI	2148491
PubMed	22151743
RGD	67379
UniProt	P09110
Wiki	ACAA1

Acetyl Coenzyme A Acyltransferase 1 is 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.Peroxisomal thiolase is markedly induced (at the level of transcription) by various hypolipidemic compounds in parallel with the other two enzymes of the peroxisomal beta-oxidation system.
The final step of beta oxidation is the cleavage of 3-ketoacyl CoA by the thiol group of another molecule of CoA. This reaction is catalyzed by Acetyl-CoA C-acyltransferase (or Β-ketothiolase). The thiol is inserted between C-2 and C-3, which yields an acetyl CoA molecule and an acyl CoA molecule, which is two carbons shorter.

Reference

"Nucleotide sequence of human peroxisomal 3-oxoacyl-CoA thiolase."
Nucleic Acids Res. 16:10369-10369(1988) [PubMed] [Europe PMC] [Abstract]

"Complete cDNA sequence of human foetal liver peroxisomal 3-oxoacyl-CoA thiolase."
Nucleic Acids Res. 17:3588-3588(1989) [PubMed] [Europe PMC] [Abstract]

"Characterization of the gene encoding human peroxisomal 3-oxoacyl-CoA thiolase (ACAA). No large DNA rearrangement in a thiolase-deficient patient."
Biochim. Biophys. Acta 1090:43-51(1991) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence, annotation and analysis of human chromosome 3."
Nature 440:1194-1198(2006) [PubMed] [Europe PMC] [Abstract]

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"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Crystal structure of human peroxisomal acetyl-COA acyl transferase 1 (ACAA1)."
Submitted (OCT-2006) to the PDB data bank