X-ray sensitivity of fifty-three human diploid fibroblast cell strains from patients with characterized genetic disorders.

TLDR

Clonogenic survival assays were used to assess the X‑irradiation response of 53 human diploid fibroblast strains. The Do values ranged from 43 to 168 rads, normal strains averaged 140–152 rads and three‑quarters of all strains fell within 128–164 rads, while strains from ataxia telangiectasia, progeria, retinoblastoma, and partial trisomy 13 were radiosensitive, and strains from other malignancy‑predisposing conditions showed no marked radiosensitivity.

Abstract

The in vitro response of 53 human diploid fibroblast strains to X-irradiation was studied using a clonogenic survival assay. The strains, derived from patients with a variety of characterized clinical conditions, most with a genetic component, ranged in Do (a measure of the slope of the survival curve) from 43 to 168 rads. The mean Do's of six strains from normal individuals was 140 to 152 rads, with an overall range, based on the extremes of their standard errors, of 128 to 164 rads. Three-quarters of the strains studied fell within this range. Strains identified as sensitive came from patients with ataxia telangiectasia, progeria, the two genetic forms of retinoblastoma, and partial trisomy of chromosome 13. No marked radiosensitivity was found among strains derived from patients with a number of other conditions associated with a predisposition to malignancy.