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Proteasomes in Distal Myopathy with Rimmed Vacuoles
Internal medicine (Tokyo. 1992)
In a previous report we suggested that muscle fibers in distal myopathywith rimmedvacuoles (DMRV) were degraded by both lysosomal proteolysis (cathepsins) and Ca2+-dependent, nonlysosomal proteolysis (calpain). Given recent evidence of abnormal ubiquitin accumulation in rimmed vacuoles, we examined the role of the ATP-ubiquitin-dependent proteolytic pathway (proteasomes) in myofiber degradation in this myopathy. Immunohistochemically, proteasomes (26S) were located in the cytoplasm in normaldoi:10.2169/internalmedicine.37.746 fatcat:sihlgw2wpnhn5dbxd2yvnyhyj4