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KMID : 0860920130150010006
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Journal of the Korean Association EMG-Electrodiagnostic Medicine
2013 Volume.15 No. 1 p.6 ~ p.10
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Immunopathologic Change of Neuromuscular Junction in Myasthenia Gravis
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Kim Jee-Young
Park Kee-Duk
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Abstract
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The most essential phenomenon of myasthenia gravis (MG) is the impaired neuromuscular transmission caused by autoantibodies directed to proteins in the postsynaptic muscle membrane of the neuromuscular junction (NMJ). It is a highly specialized structure, which is designed to facilitate a chemical transmission from a motor nerve to a skeletal muscle. The integrity and efficiency of NMJ is attributed to a systematic interaction between acetylcholine receptor (AChR) and other synaptic proteins. Immunolopathologic process induced by autoantibodies attacks other AChR-associated synaptic proteins as well as the AChRs. Although the loss of the AChRs is a key feature of MG, the damage of AChR-associated synaptic proteins aggravates the pathologic process and delays the repair process. Therefore, ideally, the goal of therapy should be to improve the potential restoration process of NMJ as well as discontinue the abnormal autoimmune process.
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KEYWORD
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Myasthenia gravis, Neuromuscular junction, Autoimmune diseases, Autoantibodies, Proteins
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