{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T17:27:56Z","timestamp":1791912476924,"version":"4.3.4"},"reference-count":81,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,12,21]],"date-time":"2017-12-21T00:00:00Z","timestamp":1513814400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100006396","name":"Alexion Pharmaceuticals","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006396","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of the New York Academy of Sciences"],"published-print":{"date-parts":[[2018,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Generalized myasthenia gravis (gMG) is a rare autoimmune disorder characterized by skeletal muscle weakness caused by disrupted neurotransmission at the neuromuscular junction (NMJ). Approximately 74\u201388% of patients with gMG have acetylcholine receptor (AChR) autoantibodies. Complement plays an important role in innate and antibody\u2010mediated immunity, and activation and amplification of complement results in the formation of membrane attack complexes (MACs), lipophilic proteins that damage cell membranes. The role of complement in gMG has been demonstrated in animal models and patients. Studies in animals lacking specific complement proteins have confirmed that MAC formation is required to induce experimental autoimmune MG (EAMG) and NMJ damage. Complement inhibition in EAMG models can prevent disease induction and reverse its progression. Patients with anti\u2010AChR\n                    <jats:sup>+<\/jats:sup>\n                    MG have autoantibodies and MACs present at NMJs. Damaged NMJs are associated with more severe disease, fewer AChRs, and MACs in synaptic debris. Current MG therapies do not target complement directly. Eculizumab is a humanized monoclonal antibody that inhibits cleavage of complement protein C5, preventing MAC formation. Eculizumab treatment improved symptoms compared with placebo in a phase II study in patients with refractory gMG. Direct complement inhibition could preserve NMJ physiology and muscle function in patients with anti\u2010AChR\n                    <jats:sup>+<\/jats:sup>\n                    gMG.\n                  <\/jats:p>","DOI":"10.1111\/nyas.13522","type":"journal-article","created":{"date-parts":[[2017,12,21]],"date-time":"2017-12-21T13:55:25Z","timestamp":1513864525000},"page":"113-128","source":"Crossref","is-referenced-by-count":210,"title":["Myasthenia gravis: the role of complement at the neuromuscular junction"],"prefix":"10.1111","volume":"1412","author":[{"suffix":"Jr.","given":"James F.","family":"Howard","sequence":"first","affiliation":[{"name":"Department of Neurology University of North Carolina  Chapel Hill North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,12,21]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2014.01.003"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrneurol.2016.44"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.12688\/f1000research.8206.1"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.48.12.1246"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880150616"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI29894"},{"key":"e_1_2_10_8_1","first-page":"267","article-title":"Immune complexes (IgG and C3) at the motor endplate in myasthenia gravis: ultrastructural and light microscopic localization and electrophysiologic correlations","volume":"52","author":"Engel A.G.","year":"1977","journal-title":"Mayo Clin. 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