Rabbit anti-Human ACOX1 Polyclonal Antibody

Uniprot Id

Q15067

Target Species

Human

Target Name

ACOX1

Target Full Name

Peroxisomal acyl-coenzyme A oxidase 1

Target Function

Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. First enzyme of the fatty acid beta-oxidation pathway.; Shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length.; Is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Is twice as active as isoform 1 against 16-hydroxy-palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl-CoA.

Target Involvement

Adrenoleukodystrophy, pseudoneonatal (Pseudo-NALD)

Target Subcellular Location

Peroxisome.

Target Protein Families

Acyl-CoA oxidase family

Target 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. Level

Target Synonyms

ACOX; ACOX1; ACOX1_HUMAN; Acyl CoA oxidase 1 palmitoyl; Acyl CoA oxidase straight chain; AOX; EC 1.3.3.6; PALMCOX; Palmitoyl CoA oxidase; Palmitoyl-CoA oxidase; Peroxisomal acyl coenzyme A oxidase 1; Peroxisomal acyl-coenzyme A oxidase 1; Peroxisomal fatty acyl CoA oxidase; SCOX; Straight chain acyl CoA oxidase; Straight-chain acyl-CoA oxidase

Target Background

The protein encoded by this gene is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. It donates electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in this gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids. Alternatively spliced transcript variants encoding different isoforms have been identified.