The Relationship of Defective Recognition of DNA Damage to Cancer in Ataxia-Telangiectasia
Ataxia-telangiectasia (A-T) is a human genetic disease that includes cancer proneness among its pathologies. Between 10 and 20% of A-T patients develop neoplastic disease, generally of the lymphoreticular system. Both the patients with A-T and their cells display hypersensitivity to ionizing radiation. This is accompanied by increased frequencies of radiation-induced chromosomal aberrations, which are almost certainly the cause of the increased cell killing (4). There is also an abnormally high frequency of spontaneous chromosomal aberrations, with a preferential involvement of chromosomes 7 and 14 in some patients (3).
KeywordsHuman Genetic Disease Aberration Frequency Premature Chromosome Condensation Preferential Involvement Chromatid Aberration
Unable to display preview. Download preview PDF.
- 3.F. Hecht and B. Kaiser-McCaw, in: “Ataxia-Telangiectasia. A Cellular and Molecular Link Between Cancer, Neuropathology, and Immune Deficiency,” B. A. Bridges and D. G. Harnden, eds., John Wiley and Sons, Chichester (1982), pp. 235–241.Google Scholar
- 6.R. B. Painter, in: “Ataxia-Telangiectasia: Genetics, Neuropathology and Immunology of a Degenerative Disease of Childhood,” R. A. Gatti and M. Swift, eds., Alan R. Liss, New York (1985), pp. 89–100.Google Scholar