Entity Details

Primary name ACOX1_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ15067
EntryNameACOX1_HUMAN
FullNamePeroxisomal acyl-coenzyme A oxidase 1
TaxID9606
Evidenceevidence at protein level
Length660
SequenceStatuscomplete
DateCreated1997-11-01
DateModified2021-06-02

Ontological Relatives

GenesACOX1

GO terms

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GOName
GO:0000038 very long-chain fatty acid metabolic process
GO:0003997 acyl-CoA oxidase activity
GO:0005504 fatty acid binding
GO:0005777 peroxisome
GO:0005778 peroxisomal membrane
GO:0005782 peroxisomal matrix
GO:0005829 cytosol
GO:0006091 generation of precursor metabolites and energy
GO:0006629 lipid metabolic process
GO:0006693 prostaglandin metabolic process
GO:0007283 spermatogenesis
GO:0008104 protein localization
GO:0009062 fatty acid catabolic process
GO:0016020 membrane
GO:0016401 palmitoyl-CoA oxidase activity
GO:0016559 peroxisome fission
GO:0019222 regulation of metabolic process
GO:0019395 fatty acid oxidation
GO:0030165 PDZ domain binding
GO:0033540 fatty acid beta-oxidation using acyl-CoA oxidase
GO:0036109 alpha-linolenic acid metabolic process
GO:0042632 cholesterol homeostasis
GO:0042803 protein homodimerization activity
GO:0047485 protein N-terminus binding
GO:0050660 flavin adenine dinucleotide binding
GO:0050665 hydrogen peroxide biosynthetic process
GO:0055088 lipid homeostasis
GO:0071949 FAD binding
GO:0140493 very long-chain fatty acid beta-oxidation

Subcellular Location

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Subcellular Location
Peroxisome

Domains

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DomainNameCategoryType
IPR002655 Acyl-CoA oxidase, C-terminalDomainDomain
IPR006091 Acyl-CoA oxidase/dehydrogenase, central domainDomainDomain
IPR009100 Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain superfamilyFamilyHomologous superfamily
IPR012258 Acyl-CoA oxidaseFamilyFamily
IPR029320 Acyl-coenzyme A oxidase, N-terminalDomainDomain
IPR034171 Peroxisomal acyl-coenzyme A oxidaseFamilyFamily
IPR036250 Acyl-CoA dehydrogenase-like, C-terminalFamilyHomologous superfamily
IPR037069 Acyl-CoA dehydrogenase/oxidase, N-terminal domain superfamilyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
264470 OMIMAdrenoleukodystrophy, pseudoneonatal (Pseudo-NALD)A peroxisomal single-enzyme disorder of fatty acid beta-oxidation, resulting in clinical manifestations that remind neonatal adrenoleukodystrophy. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty acids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning. The disease is caused by variants affecting the gene represented in this entry.
618960 OMIMMitchell syndrome (MITCH)A disorder characterized by episodic demyelination, sensorimotor polyneuropathy, and sensorineural hearing loss. The gene represented in this entry is involved in disease pathogenesis.

Drugs

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DrugNameSourceType
DB03147 Flavin adenine dinucleotideDrugbanksmall molecule
DB07930 (3R)-3-HYDROXYDODECANOIC ACIDDrugbanksmall molecule
DB09328 VayarinSwissprotbiotech