Author(s): Delaporte C, Dehaupas M, Fardeau M
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Abstract The growth "in vitro" of muscle cells from 12 patients with Duchenne muscular dystrophy (DMD) was compared with that of muscle cells from 20 age-matched controls. In the DMD explants, the lag phase (3 days) was shorter than in controls (6 days). In dissociated cells, plating efficiency (20\%) and doubling time (30 h) were identical in DMD and controls. In cultures from three DMD patients, cell clusters were occasionally observed. Myotube morphometry showed significant abnormalities in DMD cultures: the number of myotubes per field was 8.2 +/- 0.8 and 26.7 +/- 0.6 in controls, P less than 0.001; myotube length (151 +/- 20 micron) and diameter (8.2 +/- 0.9 micron) in DMD cultures were half the control values (312 +/- 46 micron and 15.6 +/- 1.2 micron, respectively, P less than 0.001). The number of nuclei per myotube in DMD was one-quarter of that in control muscle (4.0 +/- 0.2 vs 15.8 +/- 2.2, P less than 0.001). It is concluded that DMD cultures show cellular heterogeneity with the presence of fibroblasts and non-fusing myoblasts; furthermore they show delayed myoblast fusion and poor myotube differentiation.
This article was published in J Neurol Sci
and referenced in Journal of Clinical & Experimental Cardiology