Human acyl-Coenzyme A oxidase 1, palmitoyl ELISA Kit | ACOX1 elisa kit
Human Peroxisomal acyl-coenzyme A oxidase 1, ACOX1 ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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: Lipid Metabolism - alpha-linolenic acid; EC 1.3.3.6; Oxidoreductase; Lipid Metabolism - fatty acid; Lipid Metabolism - unsaturated fatty acid biosynthesis
Chromosomal Location of Human Ortholog: 17q25.1
Cellular Component: nucleoplasm; peroxisomal membrane; peroxisomal matrix; mitochondrion; intracellular membrane-bound organelle; membrane; plasma membrane; nucleolus; peroxisome; nucleus
Molecular Function: palmitoyl-CoA oxidase activity; acyl-CoA dehydrogenase activity; acyl-CoA oxidase activity; FAD binding; protein N-terminus binding; fatty acid binding; PDZ domain binding; receptor binding
Biological Process: peroxisome fission; fatty acid beta-oxidation using acyl-CoA oxidase; generation of precursor metabolites and energy; very-long-chain fatty acid metabolic process; unsaturated fatty acid metabolic process; lipid homeostasis; spermatogenesis; fatty acid oxidation; lipid metabolic process; cellular lipid metabolic process; prostaglandin metabolic process
Disease: Peroxisomal Acyl-coa Oxidase Deficiency
Research Articles on ACOX1
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Product Notes
The Human ACOX1 acox1 (Catalog #AAA9339290) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9339290 ELISA Kit recognizes Human ACOX1. It is sometimes possible for the material contained within the vial of "acyl-Coenzyme A oxidase 1, palmitoyl, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
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