Radiobiological studies in the naevoid basal cell carcinoma syndrome.
The naevoid basal cell carcinoma syndrome is described in a 43-year-old male. The response of dividing fibroblasts from this patient to the lethal effects of ionizing radiation was indistinguishable from that of a normal control: mean inactivation dose (D) = 1.74 Gy and 1.68 Gy, respectively. However, these cells were defective in the repair of potentially lethal damage (D = 2.36 Gy and 3.26 Gy, respectively). The response to radiation of Go T lymphocytes derived from the patient was similar to that of the control. The frequency of mutant circulating T lymphocytes resistant to 6-thioguanine was 17 x 10(-6) which was elevated when compared with age-matched controls. These results suggest an abnormality in the response of the fibroblasts to ionizing radiation damage.
Newton
J A
JA
Institute of Dermatology, United Medical School of Guy's Hospital, London.
Black
A K
AK
Arlett
C F
CF
Cole
J
J
D000328:Adult; D001478:Basal Cell Nevus Syndrome / Q000473:pathology*; D002470:Cell Survival / Q000528:radiation effects; D005347:Fibroblasts / Q000528:radiation effects*; D005720:Gamma Rays; D006801:Humans; D008214:Lymphocytes / Q000528:radiation effects; D008297:Male; D011829:Radiation Dosage; D012878:Skin Neoplasms / Q000473:pathology*; D014407:Tumor Cells, Cultured / Q000528:radiation effects