ACOX3 - acyl-CoA oxidase 3, pristanoyl Gene

Homo sapiens

Gene ID: 8310 | Gene type: protein coding

About ACOX3

Cytogenetic location: 4p16.1 Genomic coordinates (GRCh38): 4:8,355,305-8,440,723 (from NCBI)

This gene has 8 transcripts (splice variants), 189 orthologues and 14 paralogues. Ubiquitous expression in esophagus (RPKM 3.5), skin (RPKM 3.1) and 25 other tissues.

Summary

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 Products(10)

mRNA	Protein	Name
NM_001101667.2	NP_001095137.1	peroxisomal acyl-coenzyme A oxidase 3 isoform b
NM_001375783.1	NP_001362712.1	peroxisomal acyl-coenzyme A oxidase 3 isoform a
NM_001375784.1	NP_001362713.1	peroxisomal acyl-coenzyme A oxidase 3 isoform c
NM_001375785.1	NP_001362714.1	peroxisomal acyl-coenzyme A oxidase 3 isoform d
NM_001375786.1	NP_001362715.1	peroxisomal acyl-coenzyme A oxidase 3 isoform d
NM_001375787.1	NP_001362716.1	peroxisomal acyl-coenzyme A oxidase 3 isoform e
NM_001375788.1	NP_001362717.1	peroxisomal acyl-coenzyme A oxidase 3 isoform b
NM_001375789.1	NP_001362718.1	peroxisomal acyl-coenzyme A oxidase 3 isoform f
NM_001375790.1	NP_001362719.1	peroxisomal acyl-coenzyme A oxidase 3 isoform g
NM_003501.3	NP_003492.2	peroxisomal acyl-coenzyme A oxidase 3 isoform a

Gene Ontology

Cellular Component

Cellular Component GO Annotation	Evidence	Reference	Source
located in peroxisome	IDA IDA: Inferred from direct assay	8993592	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

ACOX3 Protein Structure

Acyl-CoA_dh_M

Acyl-CoA_dh_1

ACOX

0
200
400
600
700 a.a.

Protein Preferred Names

Protein Names

peroxisomal acyl-coenzyme A oxidase 3

BRCACox

Related Diseases

Diseases

Alias

Zellweger Syndrome

Cerebrohepatorenal Syndrome	Zellweger Leukodystrophy
Zs	Congenital Iron Overload
Chr	Zws
Severe Pbd-Zsd	Severe Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

Peroxisomal Acyl-Coa Oxidase Deficiency

Pseudoneonatal Adrenoleukodystrophy	Straight-Chain Acyl-Coa Oxidase Deficiency
Pseudoadrenoleukodystrophy	PSEUDO-NALD
Pseudo-Neonatal Adrenoleukodystrophy	Acyl-Coa Oxidase Deficiency
Peroxisomal Acyl-Coenzyme A Oxidase	Acyl-Coenzyme A Oxidase Deficiency
Adrenoleukodystrophy, Pseudoneonatal	Deficiency, Peroxisomal Acyl-Coa Oxidase

Alpha-Methylacyl-Coa Racemase Deficiency

Amacr Deficiency

AMACRD

D-Bifunctional Protein Deficiency

Bifunctional Peroxisomal Enzyme Deficiency	17-Beta-Hydroxysteroid Dehydrogenase Iv Deficiency
Dbp Deficiency	Peroxisomal Bifunctional Enzyme Deficiency
Pbfe Deficiency	Bifunctional Enzyme Deficiency
Pseudo-Zellweger Syndrome	Zellweger-Like Syndrome
DBPD	Protein Deficiency, D-Bifunctional

Peroxisomal Biogenesis Disorder

Zellweger Spectrum Disorders	Peroxisome Biogenesis Disorder-Zellweger Syndrome Spectrum
Disorders Of Peroxisome Biogenesis	Zellweger Spectrum
Zellweger Syndrome Spectrum	Peroxisomal Biogenesis Disorders
Pbd, Zss	Pbd-Zsd
Pbd-Zss	Pbd-Zellweger Spectrum Disorder
Peroxisomal Biogenesis Disorders, Zellweger Syndrome Spectrum	Peroxisome Biogenesis Disorder
Peroxisome Biogenesis Disorder Spectrum	Peroxisome Biogenesis Disorders
Zellweger Spectrum Disorder	Hyperpipecolic Acidaemia

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS00184	ACOX3 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Felis catus	ACOX3	VGNC	VGNC:59524
Rattus norvegicus	ACOX3	RGD	RGD:69245
Bos taurus	ACOX3	VGNC	VGNC:25554
Mus musculus	ACOX3	MGD	MGI:1933156
Canis familiaris	ACOX3	VGNC	VGNC:37523
Macaca mulatta	ACOX3	VGNC	VGNC:69382

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