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COX10 Polyclonal Antibody, 100μg, (ATB-T1068)
Background: Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes heme A:farnesyltransferase, which is not a structural subunit but required for the expression of functional COX and functions in the maturation of the heme A prosthetic group of COX. This protein is predicted to contain 7-9 transmembrane domains localized in the mitochondrial inner membrane. A gene mutation, which results in the substitution of a lysine for an asparagine (N204K), is identified to be responsible for cytochrome c oxidase deficiency. In addition, this gene is disrupted in patients with CMT1A (Charcot-Marie-Tooth type 1A) duplication and with HNPP (hereditary neuropathy with liability to pressure palsies) deletion. [provided by RefSeq, Jul 2008]Product datasheet: Overview Product Description COX10 Polyclonal Antibody, 100μg, (ATB-T1068) Image Species Reactivities Human Immunogen Synthesized peptide derived from the Internal region of human COX10. Properties Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. Storage Instructions-20°C/1 year References: Cytochrome c oxidase biogenesis in a patient with a mutation in COX10 gene. Coenen MJ, et al. Ann Neurol, 2004 Oct. PMID 15455402 Mutations in COX10 result in a defect in mitochondrial heme A biosynthesis and account for multiple, early-onset clinical phenotypes associated with isolated COX deficiency. Antonicka H, et al. Hum Mol Genet, 2003 Oct 15. PMID 12928484 A mutation in the human heme A:farnesyltransferase gene (COX10 ) causes cytochrome c oxidase deficiency. Valnot I, et al. Hum Mol Genet, 2000 May 1. PMID 10767350 The human COX10 gene is disrupted during homologous recombination between the 24 kb proximal and distal CMT1A-REPs. Reiter LT, et al. Hum Mol Genet, 1997 Sep. PMID 9285799 Genomic structure and expression of the human heme A:farnesyltransferase (COX10) gene. Murakami T, et al. Genomics, 1997 May 15. PMID 9177788
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At IntelixBio, our dedication to advancing healthcare is exemplified through our unparalleled range of medical lab equipment. Each product is meticulously crafted to meet the exacting standards of modern laboratory environments, facilitating seamless integration and operation. Our commitment to quality permeates every aspect of our offerings, ensuring that laboratories can consistently rely on our medical lab equipment to deliver precise and dependable results. With a comprehensive selection that spans from basic laboratory essentials to sophisticated diagnostic instruments, IntelixBio remains the foremost choice for institutions seeking excellence in medical equipment solutions. Trust IntelixBio to elevate your laboratory capabilities with our innovative medical lab equipment, empowering you to drive progress in healthcare delivery.
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