Ataxia-telangiectasia (A-T) is a rare human autosomal recessive disease with a pleiotropic phenotype characterized by cerebellar degeneration, oculocutaneous telangiectasias, immune dysfunction, genomic instability, cancer predisposition, radiation sensitivity, and premature aging. The gene mutated in A-T, ATM (A-T, mutated), encodes a 350-370 kDa protein. The C-terminal region of the protein has extensive homology to the catalytic domain of PI-3 kinase. ATM is predominantly nuclear and localizes to cytoplasmic vesicles. ATM expression is absent or expressed at very low levels in A-T cells.

Ataxia-telangiectasia, mutated (ATM) is a protein kinase that may regulate cell cycle and the response to DNA damage. ATM is known to associate with β-adaptin and β-NAP and hence, may modulate intracellular transport of proteins and vesicles. Mouse Monoclonal Anti-ATM antibody recognizes human ATM (approx. 350kDa).

Anti-ATM antibody, Mouse monoclonal has been used in:

• immunoblotting
• immunocytochemistry
• immunoprecipitation

Endogenous ATM was immunoprecipitated from whole cell lysates using monoclonal anti-ATM (SYR6D4). Immunoprecipitates were used for an ATM kinase assay.

Mouse Monoclonal Anti-ATM antibody can be used for western blot at 1-2μg/mL using a whole cell extracts of melanoma cell lines. The antibody can also be used for microarray, immunocytochemistry and immunoprecipitation assays.

Ataxia-telangiectasia (A-T) cells exhibit hypersensitivity to ionizing radiation and multiple defects responding to DNA double-strand breaks. In addition, A-T cells exhibit a variety of cellular abnormalities in culture, including cytoskeletal defects, abnormalities in the plasma membrane and defects in intracellular signaling. ATM kinase activity is enhanced immediately after exposure of cells to DNA double strand break (DSB)-inducing agents and a fraction of it is localized to nuclear aggregates, colocalized with the phosphorylated form of histone H2AX and Nbs1 protein. It binds to β-adaptin, one of the components of the AP-2 adaptor complex, which is involved in clathrin-mediated endocytosis of receptors.

Solution 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.

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