Cells from patients with ataxia telangiectasia are abnormally sensitive to the cytotoxic effect of a tumor promoter, phorbol-12-myristate-13-acetate

Mutat Res. 1985 Apr;149(2):283-6. doi: 10.1016/0027-5107(85)90035-1.


Fibroblast strains from 6 patients with ataxia telangiectasia (A-T) were found to be markedly hypersensitive to the cytotoxic action of the tumor promoter phorbol-12-myristate-13-acetate (PMA), their D37 values being 5 times lower than those of two normal controls. Two A-T heterozygous strains were slightly hypersensitive to PMA, while a third one showed normal sensitivity. It is concluded that the DNA lesion which is critical in A-T cells is an important component of the damage caused by PMA-induced free radicals and may play a role both in the tumor-promoting activity of PMA and the cancer proneness of A-T patients.

MeSH terms

  • Ataxia Telangiectasia / genetics*
  • Cell Division / drug effects
  • Cell Survival / drug effects*
  • Cells, Cultured
  • DNA Repair / drug effects
  • Heterozygote
  • Humans
  • Phorbols / toxicity*
  • Tetradecanoylphorbol Acetate / toxicity*


  • Phorbols
  • Tetradecanoylphorbol Acetate