Expression of plectin in muscle fibers with cytoarchitectural abnormalities

Plectin is a protein belonging to the cytoskeletal anchoring system, concentrated at sites of mechanical stress in different cell types. In normal skeletal muscle, plectin is located at level of Z-discs, sarcolemma, post-synaptic membrane, and intermyofibrillar network. We investigated plectin immunocytochemistry in lobulated fibers, fibers with tubular aggregates, target fibers, central core disease and centronuclear myopathy. Thirty to forty percent of lobulated fibers had patchy increase of plectin immunoreactivity at sarcolemmal level with focal subsarcolemmal increases. Tubular aggregates revealed a low binding for plectin. Ten percent of central cores exhibited faint focal increase of plectin immunoreactivity. Target formations had a normal plectin pattern. In centronuclear myopathy, plectin immunoreactivity was increased around the centrally located nuclei in 8-12% of the fibers, at the sarcolemma of 50% of type 2 fibers, and at the membrane of small vacuoles located peripherally around the central nuclei. We postulate that plectin may play a role in the subsarcolemmal aggregation of mitochondria in the lobulated fibers, and in the central position of nuclei as well as in shape formation, positioning and moving of the vacuoles in centronuclear myopathy.