The childhood muscular dystrophies: diseases sharing a common pathogenesis of membrane instability.

@article{Mendell1995TheCM,
  title={The childhood muscular dystrophies: diseases sharing a common pathogenesis of membrane instability.},
  author={Jerry R. Mendell and Zarife Sahenk and Thomas W. Prior},
  journal={Journal of child neurology},
  year={1995},
  volume={10 2},
  pages={
          150-9
        }
}
New observations demonstrate that several childhood forms of muscular dystrophy share a common pathogenesis. In muscle, dystrophin occurs as part of a membrane complex (dystrophin-glycoprotein) linking the cytoskeleton to the basal lamina. In Duchenne muscular dystrophy, dystrophin deficiency disrupts the linkage of the integral glycoproteins of the sarcolemma and leads to muscle fiber necrosis. In severe childhood autosomal recessive muscular dystrophy, a selective deficiency of adhalin (50-kd… CONTINUE READING
BETA

Citations

Publications citing this paper.
SHOWING 1-10 OF 17 CITATIONS

Similar Papers

Loading similar papers…