细胞色素c氧化酶10抗体
规格：1mg/1ml
英文名： COX10
别名： Heme A farnesyltransferase; Heme O synthase; OTTMUSP00000006085; Protoheme IX farnesyltransferase, mitochondrial precursor; RP23-78H18.1; 2410004F01Rik; AU042636; Cytochrome c oxidase assembly protein
分子量： 49kDa
储存液：Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human COX10
交叉反应：Human, Mouse, Rat,
细胞定位：
细胞色素c氧化酶10抗体产品介绍：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] Function: Converts protoheme IX and farnesyl diphosphate to heme O. Subcellular Location: Mitochondrion membrane; Multi-pass membrane protein. DISEASE: Mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]: 细胞色素c氧化酶10抗体A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, exercise intolerance, developmental delay, delayed motor development and mental retardation. Some affected individuals manifest a fatal hypertrophic cardiomyopathy resulting in neonatal death. A subset of patients manifest Leigh syndrome. Note=The disease is caused by mutations affecting the gene represented in this entry. Leigh syndrome (LS) [MIM:256000]: An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset细胞色素c氧化酶10抗体 of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the UbiA prenyltransferase family. Gene ID: 1352 Database links: Entrez Gene: 417329 Chicken Entrez Gene: 511440 Cow Entrez Gene: 1352 Human Entrez Gene: 70383 Mouse Entrez Gene: 363617 Rat Entrez Gene: 553384 Zebrafish Omim: 602125 Human SwissProt: Q12887 Human SwissProt: Q8CFY5 Mouse Unigene: 462278 Human Unigene: 340211 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
细胞色素c氧化酶10抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 信号转导 通道蛋白 新陈代谢 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid