Muscular cell integrity is assured by a number of proteins of different structure, function and localization. Their defective synthesis of genetic background results in muscular dystrophies. In these diseases progressive damage of skeletal muscle leads to disability, and in some cases affection of respiratory muscles may be a cause of early death. There is no doubt now that proper muscle function demands undisturbed collaboration of a great number of proteins, located inside the cell as well as in sarcolemma and extracellular matrix.