New approaches to the study of human dystrophic muscle cells in culture.

Tissue culture provides a system for studying the growth and differentiation of muscle cells in a controlled environment. Several studies have been carried out on diseased muscle cells in culture in attempts to elucidate the aetiology of Duchenne muscular dystrophy (DMD) but the results were equivocal. Work in our laboratory in recent years has yielded an improved method for preparing primary muscle cell cultures from dissociated biopsies which permits the morphological and biochemical evaluation of these cultures at all stages of growth and development. Our results have shown abnormalities in cell behaviour, ultrastructure and creatine kinase synthesis. The background to these studies is reviewed. Recently we have developed a cell cloning procedure that allows the accumulation of a large number of cells from a single selected cell. We can with this technique monitor quantitative and qualitative cellular and cytochemical differences between individual cell types without the ambiguities inherent in the use of mixed cell populations. The results obtained with 4 different clonal preparations derived from dystrophic muscle have shown that a number of specific features were expressed by each of the 4 clones with respect to their growth pattern, ultrastructure, synthesis of muscle specific protein and cell surface antigen. These findings clearly illustrate the potential of these cloning procedures for studying the genetic expression of homogeneous cell populations derived from normal adult human muscle and patients with X-linked muscle disease.