ACOX1 / aox recombinant protein

Recombinant Human ACOX1 / aox Protein (His tag)

Cat. Num. AAA2545835

• Gene Names: ACOX1; ACOX; SCOX; PALMCOX
• Purity: > 92 % as determined by SDS-PAGE
• Synonyms: ACOX1 / aox; Recombinant Human ACOX1 / aox Protein (His tag); ACOX; MGC1198; PALMCOX; SCOX; ACOX1 / aox recombinant protein

• Host: Baculovirus-Insect Cells
• Purity/Purification: > 92 % as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile 20mM Tris, 500mM NaCl, pH 7.0, 20% gly, 3mM DTT
• Sequence Length: 660

• Application Notes: The recombinant human ACOX1consists of 679 amino acids and has a calculated molecular mass of 76.7 kDa. It migrates as an approximately 60 kDa band in SDS-PAGE under reducing conditions.
• Predicted N Terminal: Met
• Endotoxin: < 1.0 EU per mug of the protein as determined by the LAL method
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -70 degree C

Related Product Information for ACOX1 / aox recombinant protein

Background: Peroxisomal acyl-coenzyme A oxidase 1 (ACOX1 or AOX) is the first enzyme of the fatty acid beta-oxidation pathway and belongs to the Acyl-CoA oxidase family. Human liver peroxisomes contain two acyl-CoA oxidases, namely, palmitoyl-CoA oxidase (ACOX1/AOX) and a branched chain acyl-CoA oxidase. The palmitoyl-CoA oxidase (ACOX1/AOX) oxidizes the CoA esters of straight chain fatty acids and prostaglandins and donates electrons directly to molecular oxygen, thereby producing H2O2. Human ACOX1/AOX is a protein of 661-amino acids, including the carboxyl-terminal sequence (Ser-Lys-Leu) known as a minimal peroxisome-targeting signal. Human ACOX1/AOX, the first and rate-limiting enzyme of the peroxisomal beta-oxidation pathway, has two isoforms including ACOX1a and ACOX1b, transcribed from a single gene. The human ACOX1b isoform is more effective than the ACOX1a isoform in reversing the Acox1 null phenotype in the mouse partly because of the Substrate utilization differences.

Description: A DNA sequence encoding the human ACOX1 (AAH08767.1) (Met 1-Leu 660) was expressed, with a polyhistidine tag at the N-terminus.

NCBI and Uniprot Product Information

• NCBI GI #: 14250616
• NCBI GeneID: 51
• Molecular Weight: 70,136 Da
• NCBI Official Full Name: Acyl-Coenzyme A oxidase 1, palmitoyl
• NCBI Official Synonym Full Names: acyl-CoA oxidase 1
• NCBI Official Symbol: ACOX1
• NCBI Official Synonym Symbols: ACOX; SCOX; PALMCOX
• NCBI Protein Information: peroxisomal acyl-coenzyme A oxidase 1
• UniProt Protein Name: Peroxisomal acyl-coenzyme A oxidase 1
• UniProt Gene Name: ACOX1
• UniProt Synonym Gene Names: ACOX; AOX; SCOX

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: 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