TMEM70 Antibody

Product Specifications

Background

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.

NCBI Gene ID

54968

Swiss Prot

Q9BUB7

Accession Number

NP_060336

Host

Rabbit

Reactivity

Human, Mouse, Rat

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Primary Antibodies

Field of Research

Homeostasis

Purification

TMEM70 Antibody is affinity chromatography purified via peptide column.

Positive Control

Cat. No. 1304 - Human Liver Tissue Lysate

Concentration

1 mg/mL

Homology

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

Buffer

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

Modification

None

Shipping Conditions

Blue Ice

Storage Conditions

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.

Fragment

IgG

Symbol

TMEM70

NCBI Official Name

Transmembrane protein 70

NCBI Organism

Homo sapiens

Background Reference 01

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.

Background Reference 02

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.