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Identification
HMDB Protein ID HMDBP00146
Secondary Accession Numbers
  • 5378
Name Peroxisomal acyl-coenzyme A oxidase 1
Synonyms
  1. AOX
  2. Palmitoyl-CoA oxidase
  3. SCOX
  4. Straight-chain acyl-CoA oxidase
Gene Name ACOX1
Protein Type Unknown
Biological Properties
General Function Involved in acyl-CoA dehydrogenase activity
Specific Function Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Isoform 2 is twice as active as isoform 1 against 16-hydroxy-palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl-CoA.
Pathways
  • alpha-Linolenic acid metabolism
  • Biosynthesis of unsaturated fatty acids
  • fatty acid metabolism
  • peroxisomal fatty acid beta-oxidation
  • Peroxisome
  • PPAR signaling pathway
Reactions
Acyl-CoA + Oxygen → trans-2,3-dehydroacyl-CoA + Hydrogen peroxide details
Butyryl-CoA + FAD → FADH + (E)-but-2-enoyl-CoA details
hexadecanoyl-CoA + FAD → (2E)-Hexadecenoyl-CoA + FADH details
Octanoyl-CoA + FAD → (2E)-Octenoyl-CoA + FADH details
Lauroyl-CoA + FAD → (2E)-Dodecenoyl-CoA + FADH details
Tetradecanoyl-CoA + FAD → (2E)-Tetradecenoyl-CoA + FADH details
Hexanoyl-CoA + FAD → trans-2-Hexenoyl-CoA + FADH details
Decanoyl-CoA (n-C10:0CoA) + FAD → (2E)-Decenoyl-CoA + FADH details
OPC8-CoA + FAD → trans-2-Enoyl-OPC8-CoA + FADH details
OPC6-CoA + FAD → trans-2-Enoyl-OPC6-CoA + FADH details
OPC4-CoA + FAD → trans-2-Enoyl-OPC4-CoA + FADH details
Tetracosahexaenoyl CoA + FAD → Trans-2-all-cis-6,9,12,15,18,21-tetracosaheptaenoyl-CoA + FADH details
Tetracosapentaenoyl coenzyme A, n-6 + FAD → (2E,6Z,9Z,12Z,15Z,18Z)-Tetracosahexa-2,6,9,12,15,18-enoyl-CoA + FADH details
GO Classification
Biological Process
very long-chain fatty acid metabolic process
spermatogenesis
alpha-linolenic acid metabolic process
fatty acid beta-oxidation using acyl-CoA oxidase
peroxisome fission
fatty acid oxidation
generation of precursor metabolites and energy
positive regulation of cholesterol homeostasis
prostaglandin metabolic process
lipid homeostasis
Cellular Component
mitochondrion
peroxisomal matrix
peroxisomal membrane
Component
organelle
membrane-bounded organelle
intracellular membrane-bounded organelle
microbody
peroxisome
Function
oxidoreductase activity, acting on the ch-ch group of donors
acyl-coa dehydrogenase activity
binding
catalytic activity
nucleoside binding
purine nucleoside binding
adenyl nucleotide binding
oxidoreductase activity, acting on the ch-ch group of donors, oxygen as acceptor
acyl-coa oxidase activity
oxidoreductase activity
fad or fadh2 binding
Molecular Function
acyl-CoA oxidase activity
FAD binding
acyl-CoA dehydrogenase activity
flavin adenine dinucleotide binding
palmitoyl-CoA oxidase activity
Process
metabolic process
fatty acid catabolic process
fatty acid beta-oxidation
cellular metabolic process
organic acid metabolic process
oxoacid metabolic process
carboxylic acid metabolic process
monocarboxylic acid metabolic process
fatty acid metabolic process
oxidation reduction
Cellular Location
  1. Peroxisome
Gene Properties
Chromosome Location 17
Locus 17q25.1
SNPs ACOX1
Gene Sequence
>1983 bp
ATGAACCCGGACCTGCGCAGGGAGCGGGATTCCGCCAGCTTCAACCCGGAGCTGCTTACA
CACATCCTGGACGGCAGCCCCGAGAAAACCCGGCGCCGCCGAGAGATCGAGAACATGATC
CTGAACGACCCAGACTTCCAGCATGAGGACTTGAACTTCCTCACTCGCAGCCAGCGTTAT
GAGGTGGCTGTCAGGAAAAGTGCCATCATGGTGAAGAAGATGAGGGAGTTTGGCATCGCT
GACCCTGATGAAATTATGTGGTTTAAAAATTTTGTGCACCGAGGGCGGCCTGAGCCTCTG
GATCTTCACTTGGGCATGTTCCTGCCCACCTTGCTTCACCAGGCAACTGCGGAGCAGCAG
GAGCGCTTCTTCATGCCCGCCTGGAACTTGGAGATCATTGGCACTTATGCCCAGACAGAG
ATGGGTCATGGAACTCACCTTCGAGGCTTGGAAACCACAGCCACGTATGACCCTGAAACC
CAGGAGTTCATTCTCAACAGTCCTACTGTGACCTCCATTAAATGGTGGCCTGGTGGGCTT
GGAAAGACTTCAAATCATGCAATAGTTCTTGCCCAGCTCATCACTAAGGGGAAATGCTAT
GGATTACATGCCTTTATCGTACCTATTCGTGAAATCGGGACCCATAAGCCTTTGCCAGGA
ATTACCGTTGGTGACATCGGCCCCAAATTTGGTTATGATGAGATAGACAATGGCTACCTC
AAAATGGACAACCATCGTATTCCCAGAGAAAACATGCTGATGAAGTATGCCCAGGTGAAG
CCTGATGGCACATACGTGAAACCGCTGAGTAACAAGCTGACTTACGGGACCATGGTGTTT
GTCAGGTCCTTCCTTGTGGGAGAAGCTGCTCGGGCTCTGTCTAAGGCGTGCACCATTGCC
ATCCGATACAGCGCTGTGAGGCACCAGTCTGAAATGAAGCCAGGTGAACCAGAACCACAG
ATTTTGGATTTTCAAACCCAGCAGTATAAACTCTTTCCACTCCTGGCCACTGCCTATGCC
TTCCAGTTTGTGGGCGCATACATGAAGGAGACCTATCACCGGATTAACGAAGGCATTGGT
CAAGGGGACCTGAGTGAACTGCCTGAGCTTCATGCCCTCACCGCTGGACTGAAGGCTTTC
ACCTCCTGGACTGCAAACACTGGCATTGAAGCATGTCGGATGGCTTGTGGTGGGCATGGC
TATTCTCATTGCAGTGGTCTTCCAAATATTTATGTCAATTTCACCCCAAGCTGTACCTTT
GAGGGAGAAAACACTGTCATGATGCTCCAGACGGCTAGGTTCCTGATGAAAAGTTATGAT
CAGGTGCACTCAGGAAAGTTGGTGTGTGGCATGGTGTCCTATTTGAACGACCTGCCCAGT
CAGCGCATCCAGCCACAGCAGGTAGCAGTCTGGCCAACCATGGTGGATATCAACAGCCCC
GAAAGCCTAACCGAAGCATATAAACTCCGTGCAGCCAGATTAGTAGAAATTGCTGCAAAA
AACCTTCAAAAAGAAGTGATTCACAGAAAAAGCAAGGAGGTAGCTTGGAACCTAACTTCT
GTTGACCTTGTTCGAGCAAGTGAGGCACATTGCCACTATGTGGTAGTTAAGCTCTTTTCA
GAAAAACTCCTCAAAATTCAAGATAAAGCCATTCAAGCTGTCTTAAGGAGTTTATGTCTG
CTGTATTCTCTGTATGGAATCAGTCAGAACGCGGGGGATTTCCTTCAGGGGAGCATCATG
ACAGAGCCTCAGATTACACAAGTAAACCAGCGTGTAAAGGAGTTACTCACTCTGATTCGC
TCAGATGCTGTTGCTTTGGTTGATGCATTTGATTTTCAGGATGTGACACTTGGCTCTGTG
CTTGGCCGCTATGATGGGAATGTGTATGAAAACTTGTTTGAGTGGGCTAAGAACTCCCCA
CTGAACAAAGCAGAGGTCCACGAATCTTACAAGCACCTGAAGTCACTGCAGTCCAAGCTC
TGA
Protein Properties
Number of Residues 660
Molecular Weight 70135.205
Theoretical pI 7.607
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Peroxisomal acyl-coenzyme A oxidase 1
MNPDLRRERDSASFNPELLTHILDGSPEKTRRRREIENMILNDPDFQHEDLNFLTRSQRY
EVAVRKSAIMVKKMREFGIADPDEIMWFKKLHLVNFVEPVGLNYSMFIPTLLNQGTTAQK
EKWLLSSKGLQIIGTYAQTEMGHGTHLRGLETTATYDPETQEFILNSPTVTSIKWWPGGL
GKTSNHAIVLAQLITKGKCYGLHAFIVPIREIGTHKPLPGITVGDIGPKFGYDEIDNGYL
KMDNHRIPRENMLMKYAQVKPDGTYVKPLSNKLTYGTMVFVRSFLVGEAARALSKACTIA
IRYSAVRHQSEIKPGEPEPQILDFQTQQYKLFPLLATAYAFQFVGAYMKETYHRINEGIG
QGDLSELPELHALTAGLKAFTSWTANTGIEACRMACGGHGYSHCSGLPNIYVNFTPSCTF
EGENTVMMLQTARFLMKSYDQVHSGKLVCGMVSYLNDLPSQRIQPQQVAVWPTMVDINSP
ESLTEAYKLRAARLVEIAAKNLQKEVIHRKSKEVAWNLTSVDLVRASEAHCHYVVVKLFS
EKLLKIQDKAIQAVLRSLCLLYSLYGISQNAGDFLQGSIMTEPQITQVNQRVKELLTLIR
SDAVALVDAFDFQDVTLGSVLGRYDGNVYENLFEWAKNSPLNKAEVHESYKHLKSLQSKL
GenBank ID Protein 14250616
UniProtKB/Swiss-Prot ID Q15067
UniProtKB/Swiss-Prot Entry Name ACOX1_HUMAN
PDB IDs Not Available
GenBank Gene ID BC008767
GeneCard ID ACOX1
GenAtlas ID ACOX1
HGNC ID HGNC:119
References
General References
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  8. Fournier B, Saudubray JM, Benichou B, Lyonnet S, Munnich A, Clevers H, Poll-The BT: Large deletion of the peroxisomal acyl-CoA oxidase gene in pseudoneonatal adrenoleukodystrophy. J Clin Invest. 1994 Aug;94(2):526-31. [PubMed:8040306 ]
  9. Aoyama T, Tsushima K, Souri M, Kamijo T, Suzuki Y, Shimozawa N, Orii T, Hashimoto T: Molecular cloning and functional expression of a human peroxisomal acyl-coenzyme A oxidase. Biochem Biophys Res Commun. 1994 Feb 15;198(3):1113-8. [PubMed:8117268 ]
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