Chromosome Breakage and Sensitivity to DNA Breaking Agents in Ataxia-Telangiectasia and their Possible Association with Predisposition to Cancer

  • Yechiel Becker
  • Meira Shaham
  • Yosef Shiloh
  • Ruth Voss
Part of the NATO Advanced Science Institutes Series book series (NSSA, volume 57)


Ataxia-telangiectasia (A-T) is an autosomal recessive disease characterized by increased spontaneous chromosomal breakage and a predisposition to cancer in both A-T patients and their heterozygous relatives. We have demonstrated the presence of a clastogenic factor in plasma of A-T patients, in medium conditioned by A-T fibroblasts, and in amniotic fluid of an affected A-T fetus. Our A-T fibroblast strains were found to be as sensitive as normal strains to the cytotoxic effect of methylating carcinogens. We confirmed their hypersensitivity to ionizing radiation and found that they were also markedly sensitive to treatment with the antitumor antibiotic neocarzinostatin. Two A-T heterozygote strains had intermediate sensitivity to neocarzinostatin which was distinct from that of the homozygote and the normal cells. The intrinsic hypersensitivity to DNA-breaking agents, coupled with the presence of a clastogenic factor, may account for the high rate of chromosomal breakage in vivo and the predisposition to cancer.


Conditioned Medium Amniotic Fluid Chromosome Breakage Autosomal Recessive Disease Methyl Methane Sulfonate 
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.


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Copyright information

© Springer Science+Business Media New York 1983

Authors and Affiliations

  • Yechiel Becker
    • 1
  • Meira Shaham
    • 1
  • Yosef Shiloh
    • 1
  • Ruth Voss
    • 1
  1. 1.Departments of Molecular Virology and Human GeneticsThe Hebrew University-Hadassah Medical CenterJerusalemIsrael

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