Acyl Coenzyme A Oxidase 1, Palmitoyl (ACOX1)

ACOX; PALMCOX; SCOX; Peroxisomal Acyl-Coenzyme A Oxidase 1; Straight-chain acyl-CoA oxidase

SPN	U570
HGNC	119
MGI	1330812
PubMed	20195242
RGD	619757
UniProt	Q15067
Wiki	ACOX1

Peroxisomal acyl-coenzyme A oxidase 1 is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. It donates electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in this gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids. Alternatively spliced transcript variants encoding different isoforms have been identified.The deduced human protein sequence is 89% similar to the rat protein. Like the rat homolog, the human sequence also contains C-terminal ser-lys-leu (SKL) residues which function as a peroxisomal targeting signal. Two mRNA species, 1.9-kb and 2.1-kb, were identified in liver.

Reference

"Isolation of the human peroxisomal acyl-CoA oxidase gene: organization, promoter analysis, and chromosomal localization."
Proc. Natl. Acad. Sci. U.S.A. 91:3107-3111(1994) [PubMed] [Europe PMC] [Abstract]

"Overexpression and characterization of the human peroxisomal acyl-CoA oxidase in insect cells."
J. Biol. Chem. 270:4908-4915(1995) [PubMed] [Europe PMC] [Abstract]

"Large deletion of the peroxisomal acyl-CoA oxidase gene in pseudoneonatal adrenoleukodystrophy."
J. Clin. Invest. 94:526-531(1994) [PubMed] [Europe PMC] [Abstract]

"Molecular cloning and functional expression of a human peroxisomal acyl-coenzyme A oxidase."
Biochem. Biophys. Res. Commun. 198:1113-1118(1994) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The full-ORF clone resource of the German cDNA consortium."
BMC Genomics 8:399-399(2007) [PubMed] [Europe PMC] [Abstract]

"DNA sequence of human chromosome 17 and analysis of rearrangement in the human lineage."
Nature 440:1045-1049(2006) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Global, in vivo, and site-specific phosphorylation dynamics in signaling networks."
Cell 127:635-648(2006) [PubMed] [Europe PMC] [Abstract]

"Biochemical characterization of two functional human liver acyl-CoA oxidase isoforms 1a and 1b encoded by a single gene."
Biochem. Biophys. Res. Commun. 360:314-319(2007) [PubMed] [Europe PMC] [Abstract]

"A quantitative atlas of mitotic phosphorylation."
Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]

"Lysine acetylation targets protein complexes and co-regulates major cellular functions."
Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]

"Reversal of mouse Acyl-CoA oxidase 1 (ACOX1) null phenotype by human ACOX1b isoform."
Lab. Invest. 90:696-708(2010) [PubMed] [Europe PMC] [Abstract]

Erratum
Lab. Invest. 90:808-808(2010)

"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]

"Peroxisomal acyl-CoA oxidase deficiency."
J. Pediatr. 140:128-130(2002) [PubMed] [Europe PMC] [Abstract]

"Clinical, biochemical, and mutational spectrum of peroxisomal acyl-coenzyme A oxidase deficiency."
Hum. Mutat. 28:904-912(2007) [PubMed] [Europe PMC] [Abstract]