The role of autophagy and protein homeostasis in immune-mediated necrotizing myopathy


Myofiber necrosis in the context of an inflammatory tissue reaction is the hallmark of Immune mediated necrotizing myopathy (IMNM). We and others have previously shown, that skeletal muscle fibers of IMNM patients may show non-rimmed vacuoles and sarcoplasmic irregularities. Defective chaperone activity has been shown to lead to defective assembly of myosin and other skeletal muscle proteins. We highlight cellular stress pathways involved in protein homeostasis and macroautophagy in IMNM. Muscle biopsies from IMNM patients were analyzed by immunostaining and immunofluorescence as well as by qPCR.


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