Analysis of the aquaporin expression in skeletal muscles with neuromuscular diseases.

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Aquaporin 4 (AQP4) is known to be important not only in controlling water balance of many cell types but also serve as an autoimmune target of neuromyelitis optica. In this project, we studied AQP4 expression of skeletal muscle fibers in various neuromusclular diseases including inflammatory myopathies. In immunohistochemistry, in addition to regenerating muscle fibers and denervated muscle fibers irrespective of disease, many non necrotic fibers in inflammatory myopathies showed the decrease of AQP4 expression. In the analysis of the factors which correlates to the decrease of AQP4 expression, the deposit of membrane attack complex (C5b9) in muscle tissues was clearly correlated. Our findings indicated that the activation of complement cascade decreases the AQP4 expression in skeletal muscle fibers in inflammatory myopathies, and causes functional defect of skeletal muscle fibers in inflammatory myopathies.