{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T13:40:49Z","timestamp":1791207649698,"version":"4.1.0"},"reference-count":48,"publisher":"National Academy of Sciences","issue":"9","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2009,3,3]]},"abstract":"<jats:p>\n                    Synapse formation at the neuromuscular junction (NMJ) requires an alternatively spliced variant of agrin (Z\n                    <jats:sup>+<\/jats:sup>\n                    agrin) that is produced only by neurons. Here, we show that Nova1 and Nova2, neuron-specific splicing factors identified as targets in autoimmune motor disease, are essential regulators of Z\n                    <jats:sup>+<\/jats:sup>\n                    agrin.\n                    <jats:italic>Nova1<\/jats:italic>\n                    \/\n                    <jats:italic>Nova2<\/jats:italic>\n                    double knockout mice are paralyzed and fail to cluster AChRs at the NMJ, and breeding them with transgenic mice constitutively expressing Z\n                    <jats:sup>+<\/jats:sup>\n                    agrin in motor neurons rescued AChR clustering. Surprisingly, however, these rescued mice remained paralyzed, while electrophysiologic studies demonstrated that the motor axon and synapse were functional-spontaneous and evoked recordings revealed synaptic transmission and muscle contraction. These results point to a proximal defect in motor neuron firing in the absence of Nova and reveal a previously unsuspected role for RNA regulation in the physiologic activation of motor neurons.\n                  <\/jats:p>","DOI":"10.1073\/pnas.0813112106","type":"journal-article","created":{"date-parts":[[2009,2,12]],"date-time":"2009-02-12T21:10:20Z","timestamp":1234473020000},"page":"3513-3518","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":111,"title":["Rescuing Z\n                    <sup>+<\/sup>\n                    agrin splicing in\n                    <i>Nova<\/i>\n                    null mice restores synapse formation and unmasks a physiologic defect in motor neuron firing"],"prefix":"10.1073","volume":"106","author":[{"given":"Matteo","family":"Ruggiu","sequence":"first","affiliation":[{"name":"Laboratory of Molecular Neuro-Oncology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruth","family":"Herbst","sequence":"additional","affiliation":[{"name":"Molecular Neurobiology Program, Helen and Martin S. Kimmel Center for Biology and Medicine, Skirball Institute of Biomolecular Medicine, New York University Medical School, 540 First Avenue, New York, NY 10016; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalie","family":"Kim","sequence":"additional","affiliation":[{"name":"Molecular Neurobiology Program, Helen and Martin S. Kimmel Center for Biology and Medicine, Skirball Institute of Biomolecular Medicine, New York University Medical School, 540 First Avenue, New York, NY 10016; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marko","family":"Jevsek","sequence":"additional","affiliation":[{"name":"Molecular Neurobiology Program, Helen and Martin S. Kimmel Center for Biology and Medicine, Skirball Institute of Biomolecular Medicine, New York University Medical School, 540 First Avenue, New York, NY 10016; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John J.","family":"Fak","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Neuro-Oncology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary Anne","family":"Mann","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Columbia University, New York, NY 10027"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerald","family":"Fischbach","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Columbia University, New York, NY 10027"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven J.","family":"Burden","sequence":"additional","affiliation":[{"name":"Molecular Neurobiology Program, Helen and Martin S. 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