生物来源
mouse
质量水平
偶联物
unconjugated
抗体形式
purified immunoglobulin
抗体产品类型
primary antibodies
克隆
2D3, monoclonal
表单
buffered aqueous solution
分子量
antigen ~35.57 kDa
种属反应性
human
技术
capture ELISA: suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL
同位素/亚型
IgG2aκ
NCBI登记号
UniProt登记号
运输
dry ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... COX6B1(1340)
一般描述
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. 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)
免疫原
COX6B1 (AAH01015, 1 a.a. ~ 86 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
MAEDMETKIKNYKTAPFDSRFPNQNQTRNCWQNYLDFHRCQKAMTAKGGDISVCEWYQRVYQSLCPTSWVTDWDEQRAEGTFPGKI
Sequence
MAEDMETKIKNYKTAPFDSRFPNQNQTRNCWQNYLDFHRCQKAMTAKGGDISVCEWYQRVYQSLCPTSWVTDWDEQRAEGTFPGKI
应用
Monoclonal Anti-COX6B1 antibody produced in mouse is suitable for capture ELISA, indirect ELISA and western blot applications.
生化/生理作用
COX6B1 (cytochrome c oxidase subunit VIb polypeptide 1) is involved in the regulation of mitochondrial biogenesis. Itis involved in the transport of electron from reduced cytochrome C to molecular oxygen. In muscles, COX gene consists of striated muscle-specific regulatory motifs such as E-box, CArG, and MEF2 at the proximal promoter regions. The large hydrophobic catalytic subunit acts in the electron transfer whereas the small unit is responsible for regulation and assembly of the complex. In mammals, it exists as a monomer with 13 subunits but in the active state serves as a dimer in vivo. Deficiency of COX6B1 causes mitochondrial diseases such as encephalomyopathy, hydrocephalus and hypertropic cardiomyopathy.
外形
Solution in phosphate buffered saline, pH 7.4
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储存分类代码
10 - Combustible liquids
WGK
WGK 1
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
新产品
European journal of human genetics : EJHG, 23(2), 159-164 (2014-05-02)
Isolated cytochrome c oxidase (COX) deficiency is a prevalent cause of mitochondrial disease and is mostly caused by nuclear-encoded mutations in assembly factors while rarely by mutations in structural subunits. We hereby report a case of isolated COX deficiency manifesting
American journal of human genetics, 82(6), 1281-1289 (2008-05-24)
Cytochrome c oxidase (COX) deficiency, one of the most common respiratory-chain defects in humans, has been associated with mutations in either mitochondrial DNA genes or nucleus-encoded proteins that are not part in but promote the biogenesis of COX. Mutations of
Progress in nucleic acid research and molecular biology, 61, 309-344 (1998-09-30)
Cytochrome c Oxidase (COX) is the terminal component of the bacterial as well as the mitochondrial respiratory chain complex that catalyzes the conversion of redox energy to ATP. In eukaryotes, the oligomeric enzyme is bound to mitochondrial innermembrane with subunits
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