Molecular cytoarchitecture of a myocyte, featuring proteins involved in skeletal and cardiac myopathies.
Desmin is the main muscle IF protein. It interacts with other proteins to support myofibrils at the level of the Z disc and forms a continuous cytoskeletal IF network that maintains a spatial relationship between the contractile apparatus and other structural elements of the cell. Desmin provides maintenance of cellular integrity, force transmission, and mechanochemical signaling. Mutations in other sarcomeric and cytoskeletal proteins (plectin, myotilin, filamin C, αB-crystallin, Z band alternatively spliced PDZ-motif protein [ZASP], and BCL2-associated athanogene 3 [BAG3]) cause neuromuscular disorders. Adapted with permission from