COX10 Polyclonal Antibody
- 货号：YT1068
- 应用：WB;ELISA
- 种属：Human;Mouse
- 简介：
- >>Oxidative phosphorylation;>>Porphyrin metabolism;>>Metabolic pathways;>>Biosynthesis of cofactors;>>Thermogenesis
- 蛋白名称：
- Protoheme IX farnesyltransferase mitochondrial
- 免疫原：
- The antiserum was produced against synthesized peptide derived from human COX10. AA range:98-147
- 特异性：
- COX10 Polyclonal Antibody detects endogenous levels of COX10 protein.
- 组成：
- Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 来源：
- Polyclonal, Rabbit,IgG
- 稀释：
- WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.
- 纯化工艺：
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
- 储存：
- -15°C to -25°C/1 year(Do not lower than -25°C)
- 其他名称：
- COX10;Protoheme IX farnesyltransferase; mitochondrial;Heme O synthase
- 背景：
- 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 lys
- 功能：
- disease:Defects in COX10 are a cause of cytochrome c oxidase deficiency (COX deficiency) [MIM:220110]. COX deficiency is a clinically heterogeneous disorder. The clinical features are ranging from isolated myopathy to severe multisystem disease, with onset from infancy to adulthood.,disease:Defects in COX10 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.,function:Converts protoheme IX and farnesyl diphosphate to heme O.,similarity:Belongs to the ubiA prenyltransferase family.,
- 细胞定位：
- Mitochondrion membrane; Multi-pass membrane protein.
- Western blot analysis of mouse-kidney mouse-brain Hela KB 293T lysis using COX10 antibody. Antibody was diluted at 1:1000
- Western blot analysis of lysate from HeLa cells, using COX10 antibody.
