{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T23:29:48Z","timestamp":1792798188231,"version":"4.5.0"},"reference-count":86,"publisher":"Wiley","issue":"13","license":[{"start":{"date-parts":[[2014,7,1]],"date-time":"2014-07-01T00:00:00Z","timestamp":1404172800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"crossref","award":["570930"],"award-info":[{"award-number":["570930"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Brain Foundation","id":[{"id":"https:\/\/ror.org\/01e3kxx79","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Beeren Foundation"},{"name":"Australian Myasthenic Association"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Journal of Physiology"],"published-print":{"date-parts":[[2014,7]]},"abstract":"<jats:sec>\n                    <jats:title>Key points<\/jats:title>\n                    <jats:p>\n                      <jats:list list-type=\"bullet\">\n                        <jats:list-item>\n                          <jats:p>Myasthenic anti\u2010muscle\u2010specific\u2010kinase (MuSK) IgG was injected into mice to study its effect upon the MuSK signalling pathway and the homeostasis of postsynaptic acetylcholine receptor packing at the neuromuscular junction.<\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>Densities of MuSK, activated Src kinase, phosphorylated ACh receptors and rapsyn were all reduced at motor endplates while \u03b2\u2010dystroglycan was unaffected.<\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>Pulse\u2010labelling showed that the slow decline in junctional ACh receptor density could be explained largely by diminished retention of ACh receptors within the postsynaptic membrane scaffold.<\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>The results suggest that anti\u2010MuSK IgG reduces the density of MuSK, associated tyrosine phosphorylation and retention of junctional ACh receptors within the postsynaptic membrane.<\/jats:p>\n                        <\/jats:list-item>\n                      <\/jats:list>\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Abstract<\/jats:title>\n                    <jats:p>\n                      Muscle\u2010specific kinase (MuSK) autoantibodies from myasthenia gravis patients can block the activation of MuSK\n                      <jats:italic>in vitro<\/jats:italic>\n                      and\/or reduce the postsynaptic localization of MuSK. Here we use a mouse model to examine the effects of MuSK autoantibodies upon some key components of the postsynaptic MuSK pathway and upon the regulation of junctional ACh receptor (AChR) numbers. Mice became weak after 14 daily injections of anti\u2010MuSK\u2010positive patient IgG. The intensity and area of AChR staining at the motor endplate was markedly reduced. Pulse\u2010labelling of AChRs revealed an accelerated loss of pre\u2010existing AChRs from postsynaptic AChR clusters without a compensatory increase in incorporation of (newly synthesized) replacement AChRs. Large, postsynaptic AChR clusters were replaced by a constellation of tiny AChR microaggregates. Puncta of AChR staining also appeared in the cytoplasm beneath the endplate. Endplate staining for MuSK, activated Src, rapsyn and AChR were all reduced in intensity. In the tibialis anterior muscle there was also evidence that phosphorylation of the AChR \u03b2\u2010subunit\u2010Y390 was reduced at endplates. In contrast, endplate staining for \u03b2\u2010dystroglycan (through which rapsyn couples AChR to the synaptic basement membrane) remained intense. The results suggest that anti\u2010MuSK IgG suppresses the endplate density of MuSK, thereby down\u2010regulating MuSK signalling activity and the retention of junctional AChRs locally within the postsynaptic membrane scaffold.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1113\/jphysiol.2013.270207","type":"journal-article","created":{"date-parts":[[2014,5,24]],"date-time":"2014-05-24T00:19:41Z","timestamp":1400890781000},"page":"2881-2897","source":"Crossref","is-referenced-by-count":41,"title":["Muscle\u2010specific kinase (MuSK) autoantibodies suppress the MuSK pathway and ACh receptor retention at the mouse neuromuscular junction"],"prefix":"10.1113","volume":"592","author":[{"given":"Nazanin","family":"Ghazanfari","sequence":"first","affiliation":[{"name":"Physiology and Bosch Institute University of Sydney  Sydney New South Wales 2006 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