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MuSK is a transmembrane tyrosine kinase found in the postsynaptic membrane of the neuromuscular junction. During development, MuSK serves as a signaling hub, coordinating the alignment of the pre\u2010 and postsynaptic components of the synapse. Adult mice that received repeated daily injections of IgG from anti\u2010MuSK<jats:sup>+<\/jats:sup> myasthenia gravis patients developed muscle weakness, associated with neuromuscular transmission failure. MuSK autoantibodies are predominantly of the IgG4 type. They suppress the kinase activity of MuSK and the phosphorylation of target proteins in the postsynaptic membrane. Loss of postsynaptic acetylcholine receptors is the primary cause of neuromuscular transmission failure. MuSK autoantibodies also disrupt the capacity of the motor nerve terminal to adaptively increase acetylcholine release in response to the reduced postsynaptic responsiveness to acetylcholine. The passive IgG transfer model of MuSK myasthenia gravis has been used to test candidate treatments. Pyridostigmine, a first\u2010line cholinesterase inhibitor drug, exacerbated the disease process, while 3,4\u2010diaminopyridine and albuterol were found to be beneficial in this mouse model.<\/jats:p>","DOI":"10.1111\/nyas.13513","type":"journal-article","created":{"date-parts":[[2017,11,10]],"date-time":"2017-11-10T12:46:41Z","timestamp":1510318001000},"page":"54-61","source":"Crossref","is-referenced-by-count":10,"title":["The mouse passive\u2010transfer model of MuSK myasthenia gravis: disrupted MuSK signaling causes synapse failure"],"prefix":"10.1111","volume":"1412","author":[{"given":"Nazanin","family":"Ghazanfari","sequence":"first","affiliation":[{"name":"Physiology and Bosch Institute University of Sydney  Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sofie","family":"Trajanovska","sequence":"additional","affiliation":[{"name":"Physiology and Bosch Institute University of Sydney  Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marco","family":"Morsch","sequence":"additional","affiliation":[{"name":"Physiology and Bosch Institute University of Sydney  Sydney New South Wales Australia"},{"name":"Department of Biomedical Sciences Macquarie University  Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon X.","family":"Liang","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, College of Basic Medical Sciences Liaoning Medical University  Jinzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen W.","family":"Reddel","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine Concord Hospital  Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William D.","family":"Phillips","sequence":"additional","affiliation":[{"name":"Physiology and Bosch Institute University of Sydney  Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,11,10]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1179220"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.141.6.1365"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(75)92779-8"},{"key":"e_1_2_8_5_1","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1016\/S0140-6736(86)92259-2","article-title":"Myasthenia gravis without acetylcholine receptor antibody: a distinct disease entity","volume":"327","author":"Mossman S.","year":"1986","journal-title":"Lancet"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/85520"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20061"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/0896-6273(95)90146-9"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81251-9"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/nn2026"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2008.10.002"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2008.10.006"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M007024200"},{"key":"e_1_2_8_14_1","doi-asserted-by":"crossref","first-page":"3806","DOI":"10.1523\/JNEUROSCI.21-11-03806.2001","article-title":"Src\u2010class kinases act within the agrin\/MuSK pathway to regulate acetylcholine receptor phosphorylation, cytoskeletal anchoring, and clustering","volume":"21","author":"Mohamed A.S.","year":"2001","journal-title":"J. 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