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  • Title: Antibodies against Wnt receptor of muscle-specific tyrosine kinase in myasthenia gravis.
    Author: Takamori M, Nakamura T, Motomura M.
    Journal: J Neuroimmunol; 2013 Jan 15; 254(1-2):183-6. PubMed ID: 22999188.
    Abstract:
    Muscle-specific tyrosine kinase (MuSK) antibodies are detected in a proportion of myasthenia gravis (MG) patients who are negative for acetylcholine receptor (AChR) antibodies and have prominent bulbar weakness and crises. In the MuSK ectodomains, the immunoglobulin-like 1 and 2 domains (Ig1/2) mediate the agrin-Lrp4-MuSK signaling and the cysteine-rich domain (CRD) mediates the Wnt-MuSK-Dishevelled signaling; both contribute to AChR clustering. Immunoblotting against recombinant proteins showed MuSK Ig1/2 antibodies in 33 anti-AChR-negative MG patients; 10 patients of them (30%) were additionally positive for MuSK CRD antibodies. The result suggests that MuSK antibodies have heterogeneity in their binding to functional domains of MuSK.
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