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The connection between WβC signaling and ALS. WβC signaling is upregulated in ALS. Microglia, astrocytes, and oligodendrocytes promote demyelination and inflammation and impair the neuromuscular junction in motor neurons. AchR clustering formation is important for NMJ formation. The agrin-LRP-MUSK complex is essential for the formation of NMJ. Upregulation of WβC signaling impairs the formation of Agrin-LRP4-MUSK, resulting in impaired AchR clustering in the NMJ.
