Novel Immunolocalization of α-Synuclein in Human Muscle of Inclusion-Body Myositis, Regenerating and Necrotic Muscle Fibers, and at Neuromuscular Junctions

摘要: Alpha-synuclein (alpha-syn) is an important component of neuronal and glial inclusions in brains patients with several neurodegenerative disorders. Sporadic inclusion-body myositis (s-IBM) the most common progressive muscle disease older patients. Its phenotype shows similarities Alzheimer brain. A distinct feature s-IBM pathology specific vacuolar degeneration fibers characterized by intracellular amyloid formed both amyloid-beta (Abeta) paired-helical filaments composed phosphorylated tau. We immunostained alpha-syn biopsies s-IBM, disease-control, normal Approximately 60% Abeta-positive vacuolated (VMF) contained well-defined immunoreactive antibodies against alpha-syn. In those fibers. co-localized Abeta, light microscopy, ultrastructurally. Paired-helical did not contain immunoreactivity. all biopsies, was strongly at postsynaptic region neuromuscular junctions. immunoreactivity also occurred diffusely regenerating necrotic cultured human fibers, its mRNA were expressed immunocytochemistry, immunoblots, Northern blots. Our study provides first demonstration that participates pathologic processes muscle. Therefore. function exclusive to brain diseases.