ACOX1 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. Belongs to the acyl-CoA oxidase family. 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. 3 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: EC; Lipid Metabolism - alpha-linolenic acid; Lipid Metabolism - fatty acid; Lipid Metabolism - unsaturated fatty acid biosynthesis; Oxidoreductase
Chromosomal Location of Human Ortholog: 17q25.1
Cellular Component:  cytosol; intracellular membrane-bounded organelle; nucleolus; nucleoplasm; nucleus; peroxisomal matrix; peroxisomal membrane; peroxisome; plasma membrane
Molecular Function:  acyl-CoA oxidase activity; FAD binding; PDZ domain binding; protein N-terminus binding; signaling receptor binding
Biological Process:  alpha-linolenic acid metabolic process; fatty acid beta-oxidation using acyl-CoA oxidase; fatty acid oxidation; generation of precursor metabolites and energy; lipid metabolic process; prostaglandin metabolic process; protein targeting to peroxisome; regulation of lipid metabolic process; spermatogenesis; very long-chain fatty acid metabolic process
Disease: Mitchell Syndrome; Peroxisomal Acyl-coa Oxidase Deficiency
Reference #:  Q15067 (UniProtKB)
Alt. Names/Synonyms: ACOX; ACOX1; acyl-CoA oxidase 1; acyl-CoA oxidase 1, palmitoyl; acyl-CoA oxidase, straight-chain; acyl-Coenzyme A oxidase 1, palmitoyl; AOX; MGC1198; MITCH; PALMCOX; Palmitoyl-CoA oxidase; Peroxisomal acyl-CoA oxidase 1, A chain; Peroxisomal acyl-CoA oxidase 1, B chain; Peroxisomal acyl-CoA oxidase 1, C chain; Peroxisomal acyl-coenzyme A oxidase 1; peroxisomal fatty acyl-CoA oxidase; SCOX; Straight-chain acyl-CoA oxidase
Gene Symbols: ACOX1
Molecular weight: 74,424 Da
Basal Isoelectric point: 8.35  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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