ACOX1, Polyclonal Antibody

Peroxisomal acyl-coenzyme A oxidase 1; Palmitoyl-CoA oxidase; Straight-chain acyl-CoA oxidase; SCOX; ACOX1; ACOX

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

>95%, Protein G purified

Liquid

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-ACOX1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

ELISA (EIA)

70,136 Da

acyl-CoA oxidase 1

peroxisomal acyl-coenzyme A oxidase 1

Peroxisomal acyl-coenzyme A oxidase 1

ACOX; AOX; SCOX  [Similar Products]

ACOX1_HUMAN

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. [provided by RefSeq, Jul 2008]

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. Defects in ACOX1 are the cause of adrenoleukodystrophy pseudoneonatal (Pseudo-NALD); also known as peroxisomal acyl-CoA oxidase deficiency. Pseudo-NALD is a peroxisomal single-enzyme disorder. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty cids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning. Belongs to the acyl-CoA oxidase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 1.3.3.6; 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: intracellular membrane-bound organelle; membrane; nucleolus; nucleoplasm; nucleus; peroxisomal matrix; peroxisome; plasma membrane

Molecular Function: acyl-CoA binding; acyl-CoA dehydrogenase activity; acyl-CoA oxidase activity; electron carrier activity; PDZ domain binding; protein N-terminus binding; receptor binding

Biological Process: fatty acid beta-oxidation using acyl-CoA dehydrogenase; fatty acid beta-oxidation using acyl-CoA oxidase; fatty acid oxidation; generation of precursor metabolites and energy; lipid metabolic process; prostaglandin metabolic process; very-long-chain fatty acid metabolic process

Disease: Peroxisomal Acyl-coa Oxidase Deficiency

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