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Previous Volume 333:662-664 September 7, 1995 Number 10 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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Ataxia–telangiectasia is a rare autosomal recessive disorder with diverse clinical manifestations. Patients typically come to medical attention in early childhood because of ataxia, and this is followed by progressive neuromotor degeneration, usually resulting in dependence on a wheelchair by 10 years of age. In addition to cerebellar degeneration, patients with ataxia–telangiectasia have elevated levels of alpha-fetoprotein, various immunologic deficiencies, growth retardation, premature aging, chromosomal instability, an increased frequency of lymphoreticular cancers, and hypersensitivity to ionizing radiation and radiomimetic drugs.¹ The defective gene responsible for this pleiotropic disorder (ATM) was recently identified by a multinational group led by investigators . . . [Full Text of this Article]

References

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• Fujimori, A., Okayasu, R., Ishihara, H., Yoshida, S., Eguchi-Kasai, K., Nojima, K., Ebisawa, S., Takahashi, S. (2005). Extremely Low Dose Ionizing Radiation Up-regulates CXC Chemokines in Normal Human Fibroblasts. Cancer Res. 65: 10159-10163 [Abstract] [Full Text]  
• Su, Y., Swift, M. (2001). Outcomes of Adjuvant Radiation Therapy for Breast Cancer in Women With Ataxia-Telangiectasia Mutations. JAMA 286: 2233-2234 [Full Text]  
• Fritz, E., Elsea, S. H., Patel, P. I., Meyn, M. S. (1997). Overexpression of a truncated human topoisomerase III partially corrects multiple aspects of the ataxia-telangiectasia phenotype. Proc. Natl. Acad. Sci. USA 94: 4538-4542 [Abstract] [Full Text]