产品基本信息

• 产品货号 BD-PT1068
• 别名 COX10; Protoheme IX farnesyltransferase; mitochondrial; Heme O synthase
• 产品名称 COX10 Rabbit Polyclonal Antibody
• 类别 抗原抗体
• 基因名称 COX10
• 蛋白名称 Protoheme IX farnesyltransferase mitochondrial
• Clonality Polyclonal
• 推荐应用 WB,ELISA
• 反应种属 Human,Rat,Mouse
• 浓度 1 mg/ml
• 存储缓冲液 Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
• Human Gene ID 1352
• Human Gene Link http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=1352
• Human Swissprot Link http://www.uniprot.org/uniprotkb/Q12887/entry
• Mouse Swissprot No. Q8CFY5
• Mouse Swissprot Link http://www.uniprot.org/uniprot/Q8CFY5
• 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.
• 稀释度 WB 1:500-1:2000, ELISA 1:10000.Not yet tested in other applications.
• 预测分子量 49kDa
• 运输及保存条件 -20°C/1 year
• 宿主 Rabbit
• 同种型 Rabbit,IgG
• 背景介绍 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
• 组织表达 Brain,
• 细胞定位 Mitochondrion membrane; Multi-pass membrane protein.
• 信号通路 Oxidative phosphorylation;Porphyrin and chlorophyll metabolism;
• 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.,
• 纯化 The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

图片

	Western blot analysis of lysate from HeLa cells, using COX10 antibody.
	Western blot analysis of mouse-kidney mouse-brain Hela KB 293T lysis using COX10 antibody. Antibody was diluted at 1:1000

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