{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T06:27:27Z","timestamp":1790663247802,"version":"4.1.0"},"reference-count":23,"publisher":"The Company of Biologists","license":[{"start":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T00:00:00Z","timestamp":1621296000000},"content-version":"am","delay-in-days":1598,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0"}],"funder":[{"DOI":"10.13039\/100007479","name":"Fondazione Cassa di Risparmio di Padova e Rovigo","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007479","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003500","name":"Universit\u00e0 degli Studi di Padova","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003500","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>The neuromuscular junction is exposed to different types of insults including mechanical traumas, toxins or autoimmune antibodies and, accordingly, has retained through evolution a remarkable ability to regenerate. Regeneration is driven by multiple signals that are exchanged among the cellular components of the junction. These signals are largely unknown.<\/jats:p>\n               <jats:p>Miller Fisher syndrome is a variant of Guillain-Barr\u00e9 syndrome caused by autoimmune antibodies specific for epitopes of peripheral axon terminals. Using an animal model of Miller Fisher syndrome, we recently reported that a monoclonal anti-polysialoganglioside GQ1b antibody plus complement damages nerve terminals with production of mitochondrial hydrogen peroxide, that activates Schwann cells. Several additional signaling molecules are likely to be involved in the activation of the regenerative program in these cells.<\/jats:p>\n               <jats:p>Using an in vitro cellular model consisting of co-cultured primary neurons and Schwann cells, we found that ATP is released by neurons injured by the anti-GQ1b antibody plus complement. Neuron derived ATP acts as alarm messenger for Schwann cells, where it induces the activation of intracellular pathways including calcium signaling, cyclic AMP and CREB, which in turn produce signals that promote nerve regeneration.<\/jats:p>\n               <jats:p>These results contribute to define the cross-talk taking place at the neuromuscular junction attacked by anti-gangliosides autoantibodies plus complement, functional to nerve regeneration, that are likely to be valid also for other peripheral neuropathies.<\/jats:p>","DOI":"10.1242\/dmm.027870","type":"journal-article","created":{"date-parts":[[2017,1,7]],"date-time":"2017-01-07T01:58:08Z","timestamp":1483754288000},"source":"Crossref","is-referenced-by-count":15,"title":["Schwann cells are activated by ATP released from neurons in an <i>in vitro<\/i> cellular model of Miller Fisher syndrome"],"prefix":"10.1242","author":[{"given":"Umberto","family":"Rodella","sequence":"first","affiliation":[{"name":"Department of Biomedical Sciences, University of Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuele","family":"Negro","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, University of Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"Scorzeto","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, University of Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisanna","family":"Bergamin","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, University of Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kees","family":"Jalink","sequence":"additional","affiliation":[{"name":"Division of Cell Biology, The Netherlands Cancer Institute, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7361-5688","authenticated-orcid":false,"given":"Cesare","family":"Montecucco","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, University of Padua, Italy"},{"name":"CNR Institute of Neuroscience, Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobuhiro","family":"Yuki","sequence":"additional","affiliation":[{"name":"Department of Neurology, Mishima Hospital, Niigata, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1763-2648","authenticated-orcid":false,"given":"Michela","family":"Rigoni","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, University of Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2017,1,1]]},"reference":[{"key":"2021042500123910600_DMM027870C1","doi-asserted-by":"publisher","first-page":"633","DOI":"10.1016\/j.neuron.2012.06.021","article-title":"c-Jun reprograms Schwann cells of injured nerves to generate a repair cell essential for regeneration","volume":"75","author":"Arthur-Farraj","year":"2012","journal-title":"Neuron"},{"key":"2021042500123910600_DMM027870C2","doi-asserted-by":"publisher","first-page":"1911","DOI":"10.1212\/WNL.43.10.1911","article-title":"Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barr\u00e9 syndrome: clinical and immunohistochemical studies","volume":"43","author":"Chiba","year":"1993","journal-title":"Neurology"},{"key":"2021042500123910600_DMM027870C3","doi-asserted-by":"publisher","first-page":"3901","DOI":"10.1242\/jcs.031047","article-title":"Identity, developmental restriction and reactivity of extralaminar cells capping mammalian neuromuscular junctions","volume":"121","author":"Court","year":"2008","journal-title":"J. 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