The immunofluorescent localization of antigens associated with autoimmune rippling muscles /

Abstract

Rippling muscle disease was first characterized by Torbergsen et al. in 1975. The rippling phenomena were described as stretch and percussion activated wave like contractions, that roll through the large skeletal muscles of the body. These muscle "ripples" appeared to be electrically silent (showing no motor unit action potentials). This disorder has been shown to be autosomal dominant and heterogenetic (Ricker et al., 1989). In 1996 Dr. Carl Ansevin described a patient who exhibited rippling phenomena without any family history of neuromuscular disorder. The patient's rippling muscles were associated with the onset of myasthenia gravis and both disorders were effectively treated with immunosuppressive therapies. This led to the assertion that one form of rippling muscles is related to an autoimmune response to stretch activated ca2+ channels (Ansevin et al., 1996). Our lab has shown that there are autoantibodies to high and very high molecular weight proteins in skeletal muscle, this immunoreactivity is not shown in patients with myasthenia gravis alone. Our lab has also demonstrated that rippling muscle patients also have autoantibodies to native proteins of very high molecular weight by immunoprecipitation from skeletal muscle (Walker et al., 1999). This project describes the cellular localization of rippling muscle autoantigen. Immunocytochemistry with cultured skeletal muscle myocytes show autoantibody reactivity with components of the T-tubular region of skeletal muscle.