Desminopathies in muscle disease.

  title={Desminopathies in muscle disease.},
  author={Denise Paulin and Alexis Huet and Luiza Khanamyrian and Zhigang Xue},
  journal={The Journal of pathology},
  volume={204 4},
A recently identified class of myopathies is produced by abnormal desmin, and is characterized by a disorganization of the desmin filament network, the accumulation of insoluble desmin-containing aggregates, and destructive changes in the sarcomeric organization of striated muscles. The desmin filaments interact with various other cytoskeletal proteins. The distinct clinical phenotypes are heterogeneous, with progressive skeletal myopathy, cardiomyopathy, and respiratory insufficiency as the… CONTINUE READING


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