{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,21]],"date-time":"2026-02-21T09:35:01Z","timestamp":1771666501833,"version":"3.50.1"},"reference-count":67,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2011,10,3]],"date-time":"2011-10-03T00:00:00Z","timestamp":1317600000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2011,11]]},"abstract":"<jats:sec><jats:label\/><jats:p><jats:italic>J. Neurochem.<\/jats:italic> (2011) <jats:bold>119<\/jats:bold>, 826\u2013847.<\/jats:p><\/jats:sec><jats:sec><jats:title>Abstract<\/jats:title><jats:p>Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by a gradual loss of motoneurons. The majority of ALS cases are associated with a sporadic form whose etiology is unknown. Several pieces of evidence favor autoimmunity as a potential contributor to sporadic ALS pathology. To gain understanding concerning possible antigens interacting with IgGs from sporadic ALS patients (ALS\u2013IgGs), we studied immunoreactivity against neuromuscular junction (NMJ), spinal cord and cerebellum of mice with and without the Ca<jats:sub>V<\/jats:sub>2.1 pore\u2010forming subunit of the P\/Q\u2010type voltage\u2010gated calcium (Ca<jats:sup>2+<\/jats:sup>) channel. ALS\u2013IgGs showed a strong reactivity against NMJs of wild\u2010type diaphragms. ALS\u2013IgGs also increased muscle miniature end\u2010plate potential frequency, suggesting a functional role for ALS\u2013IgGs on synaptic signaling. In support, in mice lacking the Ca<jats:sub>V<\/jats:sub>2.1 subunit ALS\u2013IgGs showed significantly reduced NMJ immunoreactivity and did not alter spontaneous acetylcholine release. This difference in reactivity was absent when comparing N\u2010type Ca<jats:sup>2+<\/jats:sup> channel wild\u2010type or null mice. These results are particularly relevant because motoneurons are known to be early pathogenic targets in ALS. Our findings add further evidence supporting autoimmunity as one of the possible mechanisms contributing to ALS pathology. They also suggest that serum autoantibodies in a subset of ALS patients would interact with NMJ proteins down\u2010regulated when P\/Q\u2010type channels are absent.<\/jats:p><\/jats:sec>","DOI":"10.1111\/j.1471-4159.2011.07462.x","type":"journal-article","created":{"date-parts":[[2011,9,1]],"date-time":"2011-09-01T04:45:29Z","timestamp":1314852329000},"page":"826-838","source":"Crossref","is-referenced-by-count":15,"title":["Amyotrophic lateral sclerosis\u2010immunoglobulins selectively interact with neuromuscular junctions expressing P\/Q\u2010type calcium channels"],"prefix":"10.1111","volume":"119","author":[{"given":"Laura E.","family":"Gonzalez","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M\u00f3nica L.","family":"Kotler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lucas G.","family":"Vattino","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eugenia","family":"Conti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ricardo C.","family":"Reisin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kirk J.","family":"Mulatz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Terrance P.","family":"Snutch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Osvaldo D.","family":"Uchitel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2011,10,3]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410360608"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1986.00520030026007"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.88.2.647"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(93)90106-9"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1080\/146608201300079418"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410400504"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamcr.2009.02.004"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/8070"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1993.tb07478.x"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(76)90527-3"},{"key":"e_1_2_7_12_1","first-page":"96","article-title":"El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. 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