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Acyl-Coenzyme A oxidase 3 also know as pristanoyl -CoA oxidase (ACOX3)is involved in the desaturation of 2-methyl branched fatty acids in peroxisomes. Unlike the rat homolog, the human gene is expressed in very low amounts in liver such that its mRNA was undetectable by routine Northern-blot analysis or its product by immunoblotting or by enzyme activity measurements. However the human cDNA encoding a 700 amino acid protein with a peroxisomal targeting C-terminal tripeptide S-K-L was isolated and is thought to be expressed under special conditions such as specific developmental stages or in a tissue specific manner in tissues that have not yet been examined. [provided by RefSeq, Jul 2008]
ACOX3 (Acyl-CoA Oxidase 3, Pristanoyl) is a Protein Coding gene. Diseases associated with ACOX3 include Zellweger Syndrome and D-Bifunctional Protein Deficiency. Among its related pathways are Linoleic acid metabolism and beta-Alanine metabolism (KEGG). Gene Ontology (GO) annotations related to this gene include signaling receptor binding and oxidoreductase activity, acting on the CH-CH group of donors. An important paralog of this gene is ACOX1.
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0003997 | acyl-CoA oxidase activity | IBA | 21873635 |
GO:0005504 | fatty acid binding | IBA | 21873635 |
GO:0016402 | pristanoyl-CoA oxidase activity | IBA | 21873635 |
GO:0016491 | oxidoreductase activity | IEA | -- |
GO:0016627 | oxidoreductase activity, acting on the CH-CH group of donors | IEA | -- |
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0005777 | peroxisome | IBA,IDA | 8993592 |
GO:0005782 | peroxisomal matrix | TAS | -- |
GO:0005829 | cytosol | TAS | -- |
GO:0016020 | membrane | HDA | 19946888 |
SuperPathway | Contained pathways | ||
---|---|---|---|
1 | Metabolism |
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2 | Fatty Acyl-CoA Biosynthesis | ||
3 | Peroxisomal lipid metabolism | ||
4 | Linoleic acid metabolism | ||
5 | beta-Alanine metabolism (KEGG) |
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0006625 | protein targeting to peroxisome | TAS | -- |
GO:0006629 | lipid metabolic process | IEA | -- |
GO:0006631 | fatty acid metabolic process | IEA | -- |
GO:0006635 | fatty acid beta-oxidation | IEA | -- |
GO:0033540 | fatty acid beta-oxidation using acyl-CoA oxidase | IBA,TAS | -- |
Name | Synonyms | Role | CAS Number | PubChem IDs | PubMed IDs | |
---|---|---|---|---|---|---|
(2E)-Decenoyl-CoA |
|
10018-95-8 |
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|||
(2E)-Dodecenoyl-CoA |
|
1066-12-2 |
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(2E)-Hexadecenoyl-CoA |
|
4460-95-1 |
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(2E)-Octenoyl-CoA |
|
10018-94-7 |
|
|||
(2E)-Tetradecenoyl-CoA |
|
38795-33-4 |
|
ExUns: | 1a | · | 1b | ^ | 2 | ^ | 3a | · | 3b | ^ | 4 | ^ | 5 | ^ | 6 | ^ | 7 | ^ | 8a | · | 8b | ^ | 9 | ^ | 10 | ^ | 11 | ^ | 12 | ^ | 13 | ^ | 14 | ^ | 15 | ^ | 16a | · | 16b | ^ | 17 | ^ | 18 | ^ | 19a | · | 19b | ^ | 20 | ^ | 21a | · |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
SP1: | - | - | - | |||||||||||||||||||||||||||||||||||||||||||||||||
SP2: | - | - | - | - | - | - | - | |||||||||||||||||||||||||||||||||||||||||||||
SP3: | ||||||||||||||||||||||||||||||||||||||||||||||||||||
SP4: | - | |||||||||||||||||||||||||||||||||||||||||||||||||||
SP5: | - | - | ||||||||||||||||||||||||||||||||||||||||||||||||||
SP6: | - | |||||||||||||||||||||||||||||||||||||||||||||||||||
SP7: | ||||||||||||||||||||||||||||||||||||||||||||||||||||
SP8: |
ExUns: | 21b | · | 21c |
---|---|---|---|
SP1: | |||
SP2: | |||
SP3: | |||
SP4: | |||
SP5: | |||
SP6: | |||
SP7: | |||
SP8: |
This gene was present in the common ancestor of eukaryotes.
Organism | Taxonomy | Gene | Similarity | Type | Details |
---|---|---|---|---|---|
Chimpanzee (Pan troglodytes) |
Mammalia | ACOX3 30 31 |
|
OneToOne | |
Dog (Canis familiaris) |
Mammalia | ACOX3 30 31 |
|
OneToOne | |
Cow (Bos Taurus) |
Mammalia | ACOX3 30 31 |
|
OneToOne | |
Mouse (Mus musculus) |
Mammalia | Acox3 30 17 31 |
|
OneToOne | |
Rat (Rattus norvegicus) |
Mammalia | Acox3 30 |
|
||
Oppossum (Monodelphis domestica) |
Mammalia | ACOX3 31 |
|
OneToOne | |
Chicken (Gallus gallus) |
Aves | ACOX3 30 31 |
|
OneToOne | |
Lizard (Anolis carolinensis) |
Reptilia | ACOX3 31 |
|
OneToOne | |
Tropical Clawed Frog (Silurana tropicalis) |
Amphibia | acox3 30 |
|
||
Zebrafish (Danio rerio) |
Actinopterygii | acox3 30 31 |
|
OneToOne | |
wufj81d02 30 |
|
||||
Rainbow Trout (Oncorhynchus mykiss) |
Actinopterygii | Omy.4390 30 |
|
||
Fruit Fly (Drosophila melanogaster) |
Insecta | CG9527 30 31 32 |
|
OneToMany | |
CG17544 31 32 |
|
OneToMany | |||
African malaria mosquito (Anopheles gambiae) |
Insecta | AgaP_AGAP007958 30 |
|
||
Worm (Caenorhabditis elegans) |
Secernentea | F58F9.7 30 31 32 |
|
OneToOne | |
Baker's yeast (Saccharomyces cerevisiae) |
Saccharomycetes | POX1 31 |
|
OneToMany | |
Thale Cress (Arabidopsis thaliana) |
eudicotyledons | ACX6 30 |
|
||
Rice (Oryza sativa) |
Liliopsida | Os06g0354500 30 |
|
SNP ID | Clinical significance and condition | Chr 04 pos | Variation | AA Info | Type |
---|---|---|---|---|---|
709676 | Benign: not provided | 8,416,438(-) | C/T | SYNONYMOUS_VARIANT,INTRON_VARIANT | |
731034 | Likely Benign: not provided | 8,392,418(-) | C/T | SYNONYMOUS_VARIANT | |
731035 | Likely Benign: not provided | 8,415,919(-) | G/A | SYNONYMOUS_VARIANT,FIVE_PRIME_UTR_VARIANT | |
740183 | Likely Benign: not provided | 8,414,860(-) | C/T | SYNONYMOUS_VARIANT | |
791970 | Benign: not provided | 8,416,371(-) | G/A | INTRON_VARIANT |
Variant ID | Type | Subtype | PubMed ID |
---|---|---|---|
esv1005399 | CNV | deletion | 20482838 |
esv1516082 | CNV | deletion | 17803354 |
esv2148081 | CNV | deletion | 18987734 |
esv2676067 | CNV | deletion | 23128226 |
esv2727113 | CNV | deletion | 23290073 |
esv2727114 | CNV | deletion | 23290073 |
esv3046818 | CNV | deletion | 24192839 |
esv34196 | OTHER | inversion | 12058347 |
esv3563378 | CNV | deletion | 23714750 |
esv3599545 | CNV | loss | 21293372 |
nsv1002794 | CNV | gain | 25217958 |
nsv1005698 | CNV | gain | 25217958 |
nsv1009011 | CNV | loss | 25217958 |
nsv4223 | CNV | insertion | 18451855 |
nsv461220 | CNV | loss | 19166990 |
nsv470013 | CNV | gain | 18288195 |
nsv470014 | CNV | loss | 18288195 |
nsv473112 | CNV | novel sequence insertion | 20440878 |
nsv524994 | CNV | loss | 19592680 |
nsv526508 | CNV | loss | 19592680 |
nsv593637 | CNV | loss | 21841781 |
nsv593638 | CNV | loss | 21841781 |
nsv593639 | CNV | loss | 21841781 |
nsv829854 | CNV | gain+loss | 17160897 |
nsv964028 | CNV | duplication | 23825009 |
Disorder | Aliases | PubMed IDs |
---|---|---|
zellweger syndrome |
|
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d-bifunctional protein deficiency |
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