|Application ||WB, IHC-P, IF, E|
|Other Accession||NP_060336, 34147498|
|Reactivity||Human, Mouse, Rat|
|Calculated MW||28969 Da|
|Application Notes||TMEM70 antibody can be used for detection of TMEM70 by Western blot at 1 µg/mL. Antibody can also be used for immunohistochemistry starting at 5 µg/mL. For immunofluorescence start at 20 µg/mL.|
|Reconstitution & Storage||TMEM70 antibody can be stored at 4℃ for three months and -20℃, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.|
|Precautions||TMEM70 Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Involved in biogenesis of mitochondrial ATP synthase.|
|Cellular Location||Mitochondrion inner membrane; Multi-pass membrane protein|
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Provided below are standard protocols that you may find useful for product applications.
TMEM70 Antibody: TMEM70 is a recently identified mitochondrial protein that is thought to play a role in the biogenesis of the ATP synthase in higher eukaryotes. Mutations in this gene result in early neonatal onset of hypotonia, hypertrophic cardiomyopathy, lactic acidos and 3-methylglutaconic aciduria (3-MGC-uria), and usually cause death within the first six weeks of life, although some patients survive much longer. Little is known of the role of TMEM70, but it is conserved across multicellular eukaryotic organisms. It contains a conserved DUF1301 domain and two putative transmembrane regions.
Cizkova A, Stranecky V, Mayr JA, et al. TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy. Nat. Genet.2008; 11:1288-90.
Honzik T, Tesarova M, Mayr JA, et al. Mitochondrial encephalocardio-myopathy with early neonatal onset due to TMEM70 mutation. Arch. Dis. Child.2010; 95:296-301.
Houstek J, Kmoch S, and Zeman J. TMEM70 protein - a novel ancillary factor of mammalian ATP synthase. Biochim. Biophys. Acta2009; 1787:529-32.
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