Title: Anti-MuSK-positive myasthenia gravis patient antibody causes aberrant activation of MuSK and disassembly of acetylcholine receptor clusters
Abstract: Purpose: Myasthenia gravis (MG) is an antibody-mediated autoimmune disease of the neuromuscular junction (NMJ) that causes impaired neuromuscular transmission and muscle weakness. Muscle Specific Kinase (MuSK) is recognized as the auto-antigen in about 10% of MG patients. Activation of MuSK by neural agrin is essential for acetylcholine receptor (AChR) clustering at the NMJ. Here we aimed to define the mechanisms by which anti-MuSK antibodies interfere with the postsynaptic AChR cluster.Conclusion: Anti-MuSK auto-antibodies cross-link and activate MuSK. This somehow causes the disassembly of large pre-existing AChR clusters.
Publication Year: 2009
Publication Date: 2009-01-01
Language: en
Type: article
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