Rabbit Anti-COX10/FITC Conjugated antibody
|别 名||Heme A farnesyltransferase; Heme O synthase; OTTMUSP00000006085; Protoheme IX farnesyltransferase, mitochondrial precursor; RP23-78H18.1; 2410004F01Rik; AU042636; Cytochrome c oxidase assembly protein; Cytochrome c oxidase subunit X; COX10_HUMAN; Protoheme IX farnesyltransferase, mitochondrial; Heme O synthase.|
|规格价格||100ul/2980元 购买 大包装/询价|
|说 明 书||100ul|
|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|
|交叉反应||Human, Mouse, Rat,|
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
|分 子 量||49kDa|
|性 状||Lyophilized or Liquid|
|免 疫 原||KLH conjugated synthetic peptide derived from human COX10|
|纯化方法||affinity purified by Protein A|
|储 存 液||0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.|
|保存条件||Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.|
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]
Converts protoheme IX and farnesyl diphosphate to heme O.
Mitochondrion membrane; Multi-pass membrane protein.
Mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]: 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 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.
Belongs to the UbiA prenyltransferase family.
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
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（COX）是线粒体呼吸链的末端成分，催化还原的细胞色素c向氧的电子转移。该组分是由3个由线粒体基因编码的催化亚基和由核基因编码的多个结构亚基组成的异构复合物。线粒体编码的亚基在电子传递中起作用，而核编码的亚基在复合物的调节和组装中起作用。该核基因编码血红素A:法尼基转移酶，它不是一个结构亚基，但表达功能COX和功能在血红素A假体COX的成熟过程中是必需的。该蛋白含有7-9个跨膜结构域，定位于线粒体内膜。一个基因突变，导致赖氨酸取代天冬酰胺（N204K），被确定为细胞色素c氧化酶缺乏的原因。此外，在CMT1A(Charcot-Marie-Tooth Type1A)复制和HNPP(易患压力性麻痹的遗传性神经病)缺失的患者中，该基因被破坏。[ RefSeq，JUL 2008 ]