The Relationship of Defective Recognition of DNA Damage to Cancer in Ataxia-Telangiectasia

  • R. B. Painter
Part of the NATO ASI Series book series (NSSA, volume 124)


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).


Human Genetic Disease Aberration Frequency Premature Chromosome Condensation Preferential Involvement Chromatid Aberration 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    M. N. Cornforth and J. S. Bedford, Science 227:1589–1591 (1985).PubMedCrossRefGoogle Scholar
  2. 2.
    K. Hansson, B. A. Kihlman, C. Tanzarella, and F. Palitti, Mutat. Res. 126:251–258 (1984).PubMedCrossRefGoogle Scholar
  3. 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
  4. 4.
    H. Nagasawa and J. B. Little, Mutat. Res. 109:297–308 (1983).PubMedCrossRefGoogle Scholar
  5. 5.
    R. B. Painter, Mutat. Res. 84:183–190 (1981).PubMedCrossRefGoogle Scholar
  6. 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
  7. 7.
    R. J. Preston, Mutat. Res. 69:71–79 (1980).PubMedCrossRefGoogle Scholar
  8. 8.
    B. K. Saha and L. J. Tolmach, Radiat. Res. 66:76–89 (1976).PubMedCrossRefGoogle Scholar
  9. 9.
    D. Scott and F. Zampetti-Bosseler, Int. J. Radiat. Biol. 42:679–683 (1982).CrossRefGoogle Scholar
  10. 10.
    P. J. Smith and M. C. Paterson, Biochim. Biophys. Acta 739:17–26 (1983).PubMedCrossRefGoogle Scholar
  11. 11.
    S. Wolff, Int. Rev. Cytol. 25:279–296 (1969).PubMedCrossRefGoogle Scholar
  12. 12.
    F. Zampetti-Bosseler and D. Scott, Int. J. Radiat. Biol. 39:547–558 (1981).CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1986

Authors and Affiliations

  • R. B. Painter
    • 1
  1. 1.Laboratory of Radiobiology and Environmental HealthUniversity of CaliforniaSan FranciscoUSA

Personalised recommendations