Anti-COX6B抗体Cytochrome c oxidase （COX）, the terminal enzyme of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen.

产品编号 byb-3935R
英文名称 Anti-COX6B抗体
中文名称 *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.
Anti-COX6B抗体 
说 明 书 0.2ml  
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 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.
分 子 量 9kDa
细胞定位 细胞浆 细胞膜 线粒体
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
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.

PubMed PubMed
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.