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Rabbit Anti-COX6B  antibody (bs-3935R)  
~~~促銷代碼KT202411~~~
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產品編號 bs-3935R
英文名稱 Rabbit Anti-COX6B  antibody
中文名稱 細胞色素C氧化酶亞基6B抗體
別    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.  
研究領域 腫瘤  細胞生物  免疫學  信號轉導  轉錄調節因子  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Cow,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 9kDa
細胞定位 細胞漿 細胞膜 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human COX6B: 2-60/86 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
P14854

Gene ID:
1340

Database links:
UniProtKB/Swiss-Prot: P14854.2

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