TMEM70 Antilichaam

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.

54968

Q9BUB7

NP_060336

Human, Mouse, Rat

Unconjugated

Primary Antibodies

Homeostasis

TMEM70 Antibody is affinity chromatography purified via peptide column.

Cat. No. 1304 - Human Liver Tissue Lysate

1 mg/mL

Predicted species reactivity based on immunogen sequence: Bovine: (93%)

TMEM70 Antibody is supplied in PBS containing 0.02% sodium azide.

None

Blue Ice

TMEM70 antibody can be stored at 4˚ C for three months and -20˚ C, 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.

IgG

TMEM70

Transmembrane protein 70

Homo sapiens

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.

TMEM70 Antibody: MC5DN2, Transmembrane protein 70, mitochondrial

ELISA, WB, IHC-P, IF

34147498

Liquid