FITC标记的细胞色素C氧化酶亚基6B抗体
产品名称： FITC标记的细胞色素C氧化酶亚基6B抗体
英文名称： Anti-COX6B/FITC
产品编号： HZ-3935R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： IF=1:50-200
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Rabbit Anti-COX6B/FITC Conjugated antibody
FITC标记的细胞色素C氧化酶亚基6B抗体
| 英文名称 | Anti-COX6B/FITC |
| 中文名称 | FITC标记的细胞色素C氧化酶亚基6B抗体 |
| 别 名 | COX 6B; COX VIb 1; COX6B; COX6B1; COXG; Cytochrome c oxidase subunit 6B1; Cytochrome c oxidase subunit VIb isoform 1; Cytochrome c oxidase subunit Vib polypeptide 1. |
| 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 免疫学 信号转导 转录调节因子 线粒体 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, |
| 产品应用 | IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 9kDa |
| 细胞定位 | 细胞膜 线粒体 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human COX6B |
| 亚 型 | IgG |
| 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. |
| 产品介绍 | background: Cytochrome c oxidase (COX), the terminal enzyme of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. It 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 be involved in the regulation and assembly of the complex. This nuclear gene encodes subunit VIb. Mutations in this gene are associated with severe infantile encephalomyopathy. Three pseudogenes COX6BP-1, COX6BP-2 and COX6BP-3 have been found on chromosomes 7, 17 and 22q13.1-13.2, respectively. [provided by RefSeq]. Function: Connects the two COX monomers into the physiological dimeric form. Subcellular Location: Mitochondrion intermembrane space. DISEASE: Defects in COX6B1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; also known as cytochrome c oxidase deficiency. 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, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome. Similarity: Belongs to the cytochrome c oxidase subunit 6B family. Database links: UniProtKB/Swiss-Prot: P14854.2 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（COX）是线粒体呼吸链的末端酶，催化从细胞色素C还原为氧的电子传递。它是由线粒体基因编码的3个催化亚基和由核基因编码的多个结构亚基组成的杂合复合体。线粒体编码亚基在电子传递中起作用，核编码亚基可能参与调节和组装复合物。该核基因编码亚基vib。该基因突变与严重婴儿脑肌病有关。在染色体7, 17和22q131-132上分别发现了三个假基因COX6BP-1、COX6BP-2和COX6BP-3。[由RefSeq提供]。