Article
Desminopathies in muscle disease.
The Journal of pathology - 1 Nov 2004
Paulin Denise, Huet Alexis, Khanamyrian Luiza, Xue Zhigang
Abstract excerpt
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...
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