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Rabbit Anti-TMEM70  antibody (bs-12664R)  
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產品編號 bs-12664R
英文名稱 Rabbit Anti-TMEM70  antibody
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.  
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 (predicted: Human)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=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 21kDa
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TMEM70: 31-130/260 
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 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.

SWISS:
Q9BUB7

Gene ID:
54968

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



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