Anti-ACOX1 Antibody Picoband® Biotin Conjugated

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.

ACOX1; ACOX; Peroxisomal acyl-coenzyme A oxidase 1; AOX; EC 1.3.3.6; Palmitoyl-CoA oxidase; Straight-chain acyl-CoA oxidase; SCOX [Cleaved into: Peroxisomal acyl-CoA oxidase 1, A chain; Peroxisomal acyl-CoA oxidase 1, B chain; Peroxisomal acyl-CoA oxidase 1, C chain]

E.coli-derived human ACOX1 recombinant protein (Position: A605-E648) . Human ACOX1shares 93.2% and 90.9% amino acid (aa) sequence identity with mouse and rat ACOX1, respectively.

1. Aoyama, T., Tsushima, K., Souri, M., Kamijo, T., Suzuki, Y., Shimozawa, N., Orii, T., Hashimoto, T. Molecular cloning and functional expression of a human peroxisomal acyl-coenzyme A oxidase. Biochem. Biophys. Res. Commun. 198: 1113-1118, 1994. 2. Carrozzo, R., Bellini, C., Lucioli, S., Deodato, F., Cassandrini, D., Cassanello, M., Caruso, U., Rizzo, C., Rizza, T., Napolitano, M. L., Wanders, R. J. A., Jakobs, C., Bruno, C., Santorelli, F. M., Dionisi-Vici, C., Bonioli, E. Peroxisomal acyl-CoA-oxidase deficiency: two new cases. Am. J. Med. Genet. 146A: 1676-1681, 2008. 3. Chung, H., Wangler, M. F., Marcogliese, P. C., Jo, J., Ravenscroft, T. A., Zuo, Z., Duraine, L., Sadeghzadeh, S., Li-Kroeger, D., Schmidt, R. E., Pestronk, A., Rosenfeld, J. A., and 23 others. Loss- or gain-of-function mutations in ACOX1 cause axonal loss via different mechanisms. Neuron 106: 589-606, 2020.