Pathogenetic Roles of Intracellular Calcium and Magnesium in Membrane-Mediated Progressive Muscle Degeneration in Duchenne Muscular Dystrophy
Duchenne muscular dystrophy (DMD) in humans is a progressively crippling X-linked recessive neuromuscular disease with no effective treatment (Rowland, 1980; Moser, 1984). It is characterized by profound biochemical (Kar and Pearson, 1976; Bertoriniet al., 1982; Bhattacharya and Crawford, 1985), electrocardiographic (Sanyal and Johnson, 1982), histopathological (Bodensteiner and Engel, 1978; Emery and Burt, 1980; Bertoriniet al., 1982, 1984), and ultrastructural (Mokri and Engel, 1975; Oberc and Engel, 1977) abnormalities of skeletal and cardiac muscle, and a 70–80% reduced life expectancy. Although a “vascular hypothesis” implicating abnormal microvasculature has been presented in the past to explain many aspects of the dystrophic pathophysiology, the most tenable mechanism for the classical muscle degeneration in DMD is now widely attributed to a generalized functional and structural defect(s) in the plasma membrane integrity of myofibers (Mokri and Engel, 1975; Schotlandet al., 1977) and erythroeytes (Araki and Mawatari, 1971).
KeywordsMuscular Dystrophy Duchenne Muscular Dystrophy Spinal Muscular Atrophy Duchenne Muscular Dystrophy Duchenne Muscular Dystrophy Patient
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