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跨膜蛋白70抗體

英文名稱 TMEM70
中文名稱 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.

說 明 書 0.2ml  
研究領域 腫瘤  細胞生物  信號轉導  跨膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat, 
產(chǎn)品應用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 29kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human TMEM70
亞    型 IgG
純化方法 affinity purified by Protein A
儲 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
PubMed PubMed
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.xy-2354R    TBX-5轉錄因子Tbx5抗體
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