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. 2021 Sep 7;97(10):e975–e987. doi: 10.1212/WNL.0000000000012463

Figure 5. Patient Immunoglobulin (Ig) Impaired Agrin-Elicited Muscle-Specific Tyrosine Kinase (MuSK) Activity and Acetylcholine Receptor (AChR) Clustering.

Figure 5

(A, B) IgG2 as the major isotype of anti-LRP4 and anti-Agrin antibodies in patient Ig. (C) Inhibition of MuSK activation by patient Ig. Phospho-MuSK elicited by agrin (C-terminal 22-KDa fragment) in myotubes treated without or with patient Ig or healthy Ig. (D) Quantitative analysis of data in C. (E–G) Inhibition of agrin-induced AChR clustering by patient Ig. Myotubes were treated without or with agrin LG3 in the presence or absence of patient or healthy Ig and assayed for AChR clusters. (E) Representative images. Scale bars: 50 μm. (F, G) Quantification of data in E. AChR clusters >4 μm in length were scored. n = 3, **p < 0.01.