Ataxia-telangiectasia (A-T) is a rare human autosomal recessive disorder caused by the mutation of ATM (A-T mutated) gene.It has defect in repair of double-strand break of DNA damage induced by ionizing radiation (IR) with losing of normal control of genomic stability in A-T patient and heterozygotes,demonstrated as the hallmarks of increased sensitivity to IR and cancer predisposition.ATM has been considered as a central controller of cellular response to DNA damage.Further study to ATM may disclose the relationship of ATM and tumorigenesis,and may provide a novel strategy for cancer prevention and therapy.