ACOX1

Acyl-CoA oxidase 1, palmitoyl

Identifiers

ACOX1; ACOX; MGC1198; PALMCOX; SCOX

External IDs

OMIM: 609751 MGI: 1330812 HomoloGene: 38299 GeneCards: ACOX1 Gene

1.3.3.6

Gene Ontology
Molecular function	• acyl-CoA dehydrogenase activity • acyl-CoA oxidase activity • acyl-CoA oxidase activity • acyl-CoA oxidase activity • protein N-terminus binding • flavin adenine dinucleotide binding
Cellular component	• cytoplasm • mitochondrion • peroxisome • peroxisomal membrane • peroxisomal matrix
Biological process	• very long-chain fatty acid metabolic process • generation of precursor metabolites and energy • lipid metabolic process • prostaglandin metabolic process • spermatogenesis • fatty acid oxidation • fatty acid beta-oxidation using acyl-CoA oxidase • fatty acid beta-oxidation using acyl-CoA oxidase • cellular lipid metabolic process • cellular lipid metabolic process
Sources: Amigo / QuickGO

RNA expression pattern

More reference expression data

Orthologs

Species

Human

Mouse

RefSeq (mRNA)

RefSeq (protein)

Location (UCSC)

Chr 17:
73.94 – 73.98 Mb

Chr 11:
116.03 – 116.06 Mb

PubMed search

[1]

[2]

Peroxisomal acyl-coenzyme A oxidase 1 is an enzyme that in humans is encoded by the ACOX1 gene.^[1]^[2]

The protein encoded by this gene 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.^[2]

References

^ Varanasi U, Chu R, Chu S, Espinosa R, LeBeau MM, Reddy JK (May 1994). "Isolation of the human peroxisomal acyl-CoA oxidase gene: organization, promoter analysis, and chromosomal localization". Proc Natl Acad Sci U S A 91 (8): 3107–11. doi:10.1073/pnas.91.8.3107. PMC 43524. PMID 8159712. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=43524.
^ ^a ^b "Entrez Gene: ACOX1 acyl-Coenzyme A oxidase 1, palmitoyl". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=51.

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Suzuki Y, Iai M, Kamei A, et al. (2002). "Peroxisomal acyl CoA oxidase deficiency". J. Pediatr. 140 (1): 128–30. doi:10.1067/mpd.2002.120511. PMID 11815777.
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ACOX1

References

Further reading