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In most MuSK MG patients the anti\u2010MuSK activity resides in the IgG4 subclass, a minor IgG component without very well\u2010defined, but presumably anti\u2010inflammatory, roles in immunity. In recent years, several animal model studies showed that anti\u2010MuSK autoantibodies can cause muscle weakness by directly affecting NMJ function and, therefore, are likely not simply bystander disease markers in MuSK MG patients. In passive transfer mice, we recently provided proof that MuSK MG patient IgG4 is severely myasthenogenic, causing functional defects at NMJs. Against the clinical, serological, and pharmacological background of MuSK MG, here we discuss the MuSK MG animal models generated by our laboratory and others that have been instrumental in elucidating the etiological and pathophysiological roles of anti\u2010MuSK antibodies.<\/jats:p>","DOI":"10.1111\/j.1749-6632.2012.06808.x","type":"journal-article","created":{"date-parts":[[2012,12,22]],"date-time":"2012-12-22T03:28:36Z","timestamp":1356146916000},"page":"114-122","source":"Crossref","is-referenced-by-count":36,"title":["Pathogenic IgG4 subclass autoantibodies in MuSK myasthenia gravis"],"prefix":"10.1111","volume":"1275","author":[{"given":"Jaap J.","family":"Plomp","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maartje G.","family":"Huijbers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silv\u00e8re M.","family":"van der Maarel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan J.","family":"Verschuuren","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,12,20]]},"reference":[{"key":"e_1_2_9_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880171208"},{"key":"e_1_2_9_3_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000129702.41868.A5"},{"key":"e_1_2_9_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.tins.2012.04.005"},{"key":"e_1_2_9_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.biocel.2010.10.008"},{"key":"e_1_2_9_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.coph.2012.02.002"},{"key":"e_1_2_9_7_2","doi-asserted-by":"publisher","DOI":"10.1242\/dev.038711"},{"key":"e_1_2_9_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2011.02.037"},{"key":"e_1_2_9_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81251-9"},{"key":"e_1_2_9_10_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.embor.7400065"},{"key":"e_1_2_9_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.mcn.2005.10.020"},{"key":"e_1_2_9_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2008.10.002"},{"key":"e_1_2_9_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/dvdy.22772"},{"key":"e_1_2_9_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2012.04.033"},{"key":"e_1_2_9_15_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature11348"},{"key":"e_1_2_9_16_2","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e32833cc968"},{"key":"e_1_2_9_17_2","doi-asserted-by":"publisher","DOI":"10.3109\/08916931003602130"},{"key":"e_1_2_9_18_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.2011.06123.x"},{"key":"e_1_2_9_19_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awn092"},{"key":"e_1_2_9_20_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2011.2393"},{"key":"e_1_2_9_21_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-011-6194-7"},{"key":"e_1_2_9_22_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22312"},{"key":"e_1_2_9_23_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20061"},{"key":"e_1_2_9_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2008.01.013"},{"key":"e_1_2_9_25_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2007.01870.x"},{"key":"e_1_2_9_26_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2009.02624.x"},{"key":"e_1_2_9_27_2","first-page":"257","article-title":"Acetylcholine receptor antibody characteristics in myasthenia gravis: I. 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