Response to ionising radiation in Duchenne muscular dystrophy

Abstract

Using lymphoblastoid cell cultures the response to gamma (γ)-radiation, was examined in 6 Duchenne muscular dystrophy (DMD) patients; 2 clinically normal males as negative controls, and 2 patients with ataxia telangiectasia (AT) showing sensitivity to ionising radiation as positive controls. In a series of experiments, cell recovery and growth at day 2 post radiation, was determined after 5 separate γ-irradiation dose levels: 50, 100, 150, 200 and 300 rads. The DMD cell strains showed a radiation dose response that was significantly greater than in cells from 2 normal males, while both DMD and normal cells were significantly less responsive than were AT-sensitive cell strains.