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Rabbit Anti-TMEM70/Cy7 Conjugated antibody (bs-12664R-Cy7)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-12664R-Cy7
英文名稱(chēng)1 Rabbit Anti-TMEM70/Cy7 Conjugated antibody
中文名稱(chēng) Cy7標(biāo)記的跨膜蛋白70抗體
別    名 FLJ20533; MC5DN2; mitochondrial; OTTHUMP00000226855; OTTHUMP00000226856; TMEM70; TMM70_HUMAN; Transmembrane protein 70; Transmembrane protein 70 mitochondrial; Transmembrane protein 70, mitochondrial.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  跨膜蛋白  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, )
產(chǎn)品應(yīng)用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 21kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TMEM70
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
This gene likely encodes a mitochondrial membrane protein. The encoded protein may play a role in biogenesis of mitochondrial ATP synthase. Mutations in this gene have been associated with neonatal mitochondrial encephalocardiomyopathy due to ATP synthase deficiency. Alternatively spliced transcript variants have been described. [provided by RefSeq, Feb 2010]

Function:
Involved in biogenesis of mitochondrial ATP synthase.

Subcellular Location:
Mitochondrion inner membrane.

DISEASE:
Defects in TMEM70 are a cause of mitochondrial encephalocardiomyopathy neonatal due to ATP synthase deficiency (MT-ATPSD) [MIM:604273]; also known as ATPase deficiency. A mitochondrial disorder with heterogeneous clinical manifestations including dysmorphic features, psychomotor retardation, hypotonia, growth retardation, cardiomyopathy, enlarged liver, hypoplastic kidneys and elevated lactate levels in urine, plasma and cerebrospinal fluid.

Similarity:
Belongs to the TMEM70 family.

Database links:

Entrez Gene: 54968 Human

Entrez Gene: 70397 Mouse

Entrez Gene: 500384 Rat

Omim: 612418 Human

SwissProt: Q9BUB7 Human

SwissProt: Q921N7 Mouse

Unigene: 106650 Human

Unigene: 194225 Mouse

Unigene: 22296 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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