细胞色素C氧化酶亚基6B抗体
规格：1mg/1ml
英文名： COX6B
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.
分子量： 9kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
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 COX6B
交叉反应：Human, Mouse, Rat, Pig, Cow, Horse, Rabbit,
细胞定位：细胞浆 细胞膜 线粒体
产细胞色素C氧化酶亚基6B抗体品介绍：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 细胞色素C氧化酶亚基6B抗体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 细胞色素C氧化酶亚基6B抗体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氧化酶亚基6B抗体产品应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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 **学 信号转导 转录调节因子 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