生物源
mouse
共軛
unconjugated
抗體表格
purified immunoglobulin
抗體產品種類
primary antibodies
無性繁殖
2D3, monoclonal
形狀
buffered aqueous solution
分子量
antigen ~35.57 kDa
物種活性
human
技術
capture ELISA: suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL
同型
IgG2aκ
NCBI登錄號
UniProt登錄號
運輸包裝
dry ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... COX6B1(1340)
一般說明
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
應用
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.
外觀
Solution in phosphate buffered saline, pH 7.4
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儲存類別代碼
10 - Combustible liquids
水污染物質分類(WGK)
WGK 1
閃點(°F)
Not applicable
閃點(°C)
Not applicable
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
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
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
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