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They target mainly the first Ig-like domain of MuSK, an epitope which is located on the extracellular part of the protein and mediates LRP4-MuSK binding (McConville et al., in vivo models of MuSK-MG lack the presynaptic adaptive increase of ACh release (quantal content) observed in AChR-MG models (Mori et al., 2012

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Its manifestations are both peripheral and central, presenting as a condition of weakness and torpor associated with a reduction in muscle strength

Schaeffer AJ, Datta NS, Fowler JE Jr, Krieger JN, Litwin MS, Nadler RB, Nickel JC, Pontari MA, Shoskes DA, Zeitlin SI, et al

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