A sensitive assay for detecting hypersensitivity to ionizing radiation in lymphoblastoid lines from patients with Duchenne muscular dystrophy and primary neuronal degenerations.

Hypersensitivity to the lethal effects of DNA-damaging agents is usually demonstrated using the classical colony-forming ability assay with cultured fibroblast lines. Based on the ability of viable cells in lymphoblastoid lines (Epstein-Barr virus-transformed B lymphocytes) to exclude the vital dye trypan blue, we have developed a more rapid survival assay which has been useful in detecting hypersensitivity to ionizing radiation in certain diseases characterized by primary degeneration of excitable tissue. We now present a complete description of this post-X-ray survival assay. We also demonstrate the suitability of both our assay and our method of data analysis for detecting hypersensitivity to ionizing radiation. This demonstration is based on a detailed analysis of assay results with lymphoblastoid lines from 28 normal donors, 3 ataxia telangiectasia (AT) patients, 2 obligate AT heterozygotes, 7 patients with diseases characterized by cellular hypersensitivity to ultraviolet radiation (UV), and 10 Duchenne muscular dystrophy (DMD) patients.