InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: ACOX1

Summary

InnateDB Protein

IDBP-69163.5

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

ACOX1

Protein Name

acyl-CoA oxidase 1, palmitoyl

Synonyms

ACOX; PALMCOX; SCOX;

Species

Homo sapiens

Ensembl Protein

ENSP00000301608

InnateDB Gene

IDBG-69159 (ACOX1)

Protein Structure

UniProt Annotation

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. {ECO:0000269PubMed:17458872, ECO:0000269PubMed:17603022}.

Subcellular Localization

Peroxisome.

Disease Associations

Adrenoleukodystrophy, pseudoneonatal (Pseudo-NALD) [MIM:264470]: 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. {ECO:0000269PubMed:11815777, ECO:0000269PubMed:17458872, ECO:0000269PubMed:8040306}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Widely expressed with highest levels of isoform 1 and isoform 2 detected in testis. Isoform 1 is expressed at higher levels than isoform 2 in liver and kidney while isoform 2 levels are higher in brain, lung, muscle, white adipose tissue and testis. Levels are almost equal in heart. {ECO:0000269PubMed:17603022, ECO:0000269PubMed:20195242}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 14 experimentally validated interaction(s) in this database.

Experimentally validated
Total	14 [view]
Protein-Protein	13 [view]
Protein-DNA	1 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003995	acyl-CoA dehydrogenase activity
GO:0003997	acyl-CoA oxidase activity
GO:0005102	receptor binding
GO:0016401	palmitoyl-CoA oxidase activity
GO:0016627	oxidoreductase activity, acting on the CH-CH group of donors
GO:0030165	PDZ domain binding
GO:0047485	protein N-terminus binding
GO:0050660	flavin adenine dinucleotide binding
GO:0071949	FAD binding

Biological Process

GO:0000038	very long-chain fatty acid metabolic process
GO:0006091	generation of precursor metabolites and energy
GO:0006629	lipid metabolic process
GO:0006631	fatty acid metabolic process
GO:0006635	fatty acid beta-oxidation
GO:0006693	prostaglandin metabolic process
GO:0008152	metabolic process
GO:0016559	peroxisome fission
GO:0019395	fatty acid oxidation
GO:0033540	fatty acid beta-oxidation using acyl-CoA oxidase
GO:0033559	unsaturated fatty acid metabolic process
GO:0036109	alpha-linolenic acid metabolic process
GO:0044255	cellular lipid metabolic process
GO:0044281	small molecule metabolic process
GO:0055088	lipid homeostasis
GO:0055114	oxidation-reduction process
GO:2000189	positive regulation of cholesterol homeostasis

Cellular Component

GO:0005777	peroxisome
GO:0005782	peroxisomal matrix
GO:0016020	membrane

Protein Structure and Domains

PDB ID

InterPro

IPR002655 Acyl-CoA oxidase, C-terminal
IPR006091 Acyl-CoA oxidase/dehydrogenase, central domain
IPR009075 Acyl-CoA dehydrogenase/oxidase C-terminal
IPR009100 Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain
IPR012258 Acyl-CoA oxidase

PFAM

PF01756
PF02770
PF00441

PRINTS

PIRSF

PIRSF000168

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q15067