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Merck

SAB1403687

Sigma-Aldrich

Monoclonal Anti-COX6B1 antibody produced in mouse

clone 2D3, purified immunoglobulin, buffered aqueous solution

別名:

COX6B, COXG

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100 μG
¥60,750

¥60,750


出荷予定日2025年4月15日



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100 μG
¥60,750

About This Item

UNSPSCコード:
12352203
NACRES:
NA.41

¥60,750


出荷予定日2025年4月15日


由来生物

mouse

品質水準

結合体

unconjugated

抗体製品の状態

purified immunoglobulin

抗体製品タイプ

primary antibodies

クローン

2D3, monoclonal

フォーム

buffered aqueous solution

分子量

antigen ~35.57 kDa

化学種の反応性

human

テクニック

capture ELISA: suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL

アイソタイプ

IgG2aκ

NCBIアクセッション番号

UniProtアクセッション番号

輸送温度

dry ice

保管温度

−20°C

ターゲットの翻訳後修飾

unmodified

遺伝子情報

human ... COX6B1(1340)

詳細

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

アプリケーション

Monoclonal Anti-COX6B1 antibody produced in mouse is suitable for capture ELISA, indirect ELISA and western blot applications.

生物化学的/生理学的作用

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.

物理的形状

無色透明のPBS溶液、pH 7.4

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保管分類コード

10 - Combustible liquids

WGK

WGK 1

引火点(°F)

Not applicable

引火点(℃)

Not applicable


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Ulla Najwa Abdulhag et al.
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
Valeria Massa et al.
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
N Lenka et al.
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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