Cultured diploid fibroblasts from patients with the nevoid basal cell carcinoma syndrome are hypersensitive to killing by ionizing radiation

Journal Article · Fri Apr 01 04:00:00 EST 1983 · Am. J. Pathol.; (United States)

OSTI ID:5853669

Chan, G L; Little, J B

Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disease. About 20% of the gene carriers studied developed medulloblastoma before the age of 5 years. Clinical follow-up of these patients, treated with radiotherapy, revealed a predisposition to radiogenic basal cell carcinomas with an unusually short latent period of 6 months to 3 years. The authors have therefore cultured skin fibroblasts from 5 NBCCS patients and measured their radiosensitivity in terms of clonogenic survival. Our results showed that, compared with 6 normal controls, the NBCCS cells were hypersensitive to X-rays. The average D0 (the inverse of the slope of the survival curve) for the NBCCS cells was 98 rads, compared with 142 rads for the normal controls and 44 rads for an ataxia telangiectasia (AT) strain. The average D10 values (the dose required to reduce survival to 10%) were 258, 351, and 123 rads for the NBCCS, normal, and AT strains, respectively. Unscheduled DNA synthesis measurements showed that NBCCS cells were not defective in excision repair of X-ray-damaged DNA. Pulse labeling index measurements showed that NBCCS cells were abnormally inhibited in the initiation of DNA synthesis following X-irradiation. The mechanisms underlying the radiosensitivity of NBCCS differ in several respects from those of AT. NBCCS appears to be potentially a useful model for studying the cellular processes that are important in radiation carcinogenesis.

• Research Organization: Laboratory of Radiobiology, Harvard School of Public Health, Boston, Massachusetts
• OSTI ID: 5853669
• Journal Information: Am. J. Pathol.; (United States), Journal Name: Am. J. Pathol.; (United States) Vol. 111:1; ISSN AJPAA
• Country of Publication: United States
• Language: English

Related Subjects

560121* -- Radiation Effects on Cells-- External Source-- (-1987)
63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.
ANIMAL CELLS
CARCINOMAS
CELL CULTURES
CELL KILLING
CONNECTIVE TISSUE CELLS
DISEASES
ELECTROMAGNETIC RADIATION
FIBROBLASTS
IONIZING RADIATIONS
NEOPLASMS
RADIATIONS
RADIOSENSITIVITY
SOMATIC CELLS
TUMOR CELLS
X RADIATION