{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T17:28:32Z","timestamp":1791912512399,"version":"4.3.4"},"reference-count":365,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2019,7,2]],"date-time":"2019-07-02T00:00:00Z","timestamp":1562025600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002428","name":"Austrian Science Fund","doi-asserted-by":"publisher","award":["P28485-B27, T996-B30"],"award-info":[{"award-number":["P28485-B27, T996-B30"]}],"id":[{"id":"10.13039\/501100002428","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction (NMJ). Autoantibodies target key molecules at the NMJ, such as the nicotinic acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (Lrp4), that lead by a range of different pathogenic mechanisms to altered tissue architecture and reduced densities or functionality of AChRs, reduced neuromuscular transmission, and therefore a severe fatigable skeletal muscle weakness. In this review, we give an overview of the history and clinical aspects of MG, with a focus on the structure and function of myasthenic autoantigens at the NMJ and how they are affected by the autoantibodies\u2019 pathogenic mechanisms. Furthermore, we give a short overview of the cells that are implicated in the production of the autoantibodies and briefly discuss diagnostic challenges and treatment strategies.<\/jats:p>","DOI":"10.3390\/cells8070671","type":"journal-article","created":{"date-parts":[[2019,7,2]],"date-time":"2019-07-02T12:11:17Z","timestamp":1562069477000},"page":"671","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":181,"title":["Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture"],"prefix":"10.3390","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0567-021X","authenticated-orcid":false,"given":"Inga","family":"Koneczny","sequence":"first","affiliation":[{"name":"Institute of Neurology, Medical University of Vienna, 1090 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7764-5363","authenticated-orcid":false,"given":"Ruth","family":"Herbst","sequence":"additional","affiliation":[{"name":"Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, 1090 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2019,7,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Carr, A.S., Cardwell, C.R., McCarron, P.O., and McConville, J. (2010). A systematic review of population based epidemiological studies in Myasthenia Gravis. BMC Neurol., 10.","DOI":"10.1186\/1471-2377-10-46"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1136\/jnnp.2006.102517","article-title":"Epidemiology of myasthenia gravis with anti-muscle specific kinase antibodies in The Netherlands","volume":"78","author":"Niks","year":"2007","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1007\/s00508-012-0258-2","article-title":"Epidemiology of myasthenia gravis in Austria: Rising prevalence in an ageing society","volume":"124","author":"Cetin","year":"2012","journal-title":"Wien. Klin. Wochenschr."},{"key":"ref_4","first-page":"168","article-title":"Myasthenia gravis","volume":"Volume 5","author":"Ratcliffe","year":"2016","journal-title":"Encyclopedia of Immunobiology"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1212\/WNL.38.1.138","article-title":"Neonatal myasthenia gravis: A new clinical and immunologic appraisal on 30 cases","volume":"38","author":"Morel","year":"1988","journal-title":"Neurology"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/0896-8411(91)90017-7","article-title":"Anti-acetylcholine receptor antibodies in neonatal myasthenia gravis: Heterogeneity and pathogenic significance","volume":"4","author":"Eymard","year":"1991","journal-title":"J. Autoimmun."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1016\/j.nmd.2008.03.006","article-title":"Severe neonatal myasthenia due to maternal anti-MuSK antibodies","volume":"18","author":"Behin","year":"2008","journal-title":"Neuromuscul. Disord."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/S0140-6736(95)92652-6","article-title":"Arthrogryposis multiplex congenita with maternal autoantibodies specific for a fetal antigen","volume":"346","author":"Vincent","year":"1995","journal-title":"Lancet"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2010","DOI":"10.1212\/01.wnl.0000336929.38733.7a","article-title":"Fetal acetylcholine receptor inactivation syndrome and maternal myasthenia gravis","volume":"71","author":"Oskoui","year":"2008","journal-title":"Neurology"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"546","DOI":"10.1016\/j.nmd.2012.01.002","article-title":"Fetal acetylcholine receptor inactivation syndrome and maternal myasthenia gravis: A case report","volume":"22","author":"Bertini","year":"2012","journal-title":"Neuromuscul. Disord."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e57","DOI":"10.1212\/NXI.0000000000000057","article-title":"Fetal acetylcholine receptor inactivation syndrome: A myopathy due to maternal antibodies","volume":"2","author":"Hacohen","year":"2015","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/j.cca.2015.01.042","article-title":"An update on laboratory diagnosis in myasthenia gravis","volume":"444","author":"Oger","year":"2015","journal-title":"Clin. Chim. Acta"},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Sanders, D.B., Arimura, K., Cui, L., Ertas, M., Farrugia, M.E., Gilchrist, J., Kouyoumdjian, J.A., Padua, L., Pitt, M., and Stalberg, E. (2019). Guidelines for single fiber EMG. Clin. Neurophysiol.","DOI":"10.1016\/j.clinph.2019.04.005"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1002\/mus.20435","article-title":"Single-fiber EMG with a concentric needle electrode: Validation in myasthenia gravis","volume":"33","author":"Sarrigiannis","year":"2006","journal-title":"Muscle Nerve"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1159\/000324915","article-title":"Repetitive nerve stimulation of facial and hypothenar muscles: Relative sensitivity in different myasthenia gravis subgroups","volume":"65","author":"Zambelis","year":"2011","journal-title":"Eur. Neurol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1111\/nyas.13518","article-title":"Myasthenia gravis with antibodies to MuSK: An update","volume":"1412","author":"Evoli","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1038\/s41572-019-0079-y","article-title":"Myasthenia gravis","volume":"5","author":"Gilhus","year":"2019","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1016\/j.ncl.2018.01.010","article-title":"Diagnosis of Myasthenia Gravis","volume":"36","author":"Pasnoor","year":"2018","journal-title":"Neurol. Clin."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1111\/nyas.13537","article-title":"Developing treatment guidelines for myasthenia gravis","volume":"1412","author":"Sanders","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1038\/2071097a0","article-title":"Release of acetylcholine from a nerve terminal by electric pulses of variable strength and duration","volume":"207","author":"Katz","year":"1965","journal-title":"Nature"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1113\/jphysiol.1952.sp004735","article-title":"Spontaneous subthreshold activity at motor nerve endings","volume":"117","author":"Fatt","year":"1952","journal-title":"J. Physiol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1093\/brain\/115.2.451","article-title":"Structure and function of neuromuscular junctions in the vastus lateralis of man. A motor point biopsy study of two groups of patients","volume":"115","author":"Slater","year":"1992","journal-title":"Brain"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1016\/S0301-0082(00)00055-1","article-title":"Safety factor at the neuromuscular junction","volume":"64","author":"Wood","year":"2001","journal-title":"Prog. Neurobiol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1016\/j.ncl.2018.01.001","article-title":"Nature and Action of Antibodies in Myasthenia Gravis","volume":"36","author":"Ruff","year":"2018","journal-title":"Neurol. Clin."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1113\/jphysiol.1964.sp007495","article-title":"An Electrophysiological Investigation of Neuromuscular Transmission in Myasthenia Gravis","volume":"174","author":"Elmqvist","year":"1964","journal-title":"J. Physiol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.jneuroim.2008.04.038","article-title":"How myasthenia gravis alters the safety factor for neuromuscular transmission","volume":"201\u2013202","author":"Ruff","year":"2008","journal-title":"J. Neuroimmunol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1111\/j.1749-6632.1987.tb51322.x","article-title":"The electrodiagnosis of myasthenia gravis","volume":"505","author":"Sanders","year":"1987","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1002\/ana.410370513","article-title":"Acetylcholine release in myasthenia gravis: Regulation at single end-plate level","volume":"37","author":"Plomp","year":"1995","journal-title":"Ann. Neurol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1146\/annurev-physiol-021014-071740","article-title":"Homeostatic control of presynaptic neurotransmitter release","volume":"77","author":"Davis","year":"2015","journal-title":"Annu. Rev. Physiol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"828","DOI":"10.1523\/JNEUROSCI.3786-15.2016","article-title":"Reversible Recruitment of a Homeostatic Reserve Pool of Synaptic Vesicles Underlies Rapid Homeostatic Plasticity of Quantal Content","volume":"36","author":"Wang","year":"2016","journal-title":"J. Neurosci."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1945","DOI":"10.1212\/01.WNL.0000128048.23930.1D","article-title":"Are MuSK antibodies the primary cause of myasthenic symptoms?","volume":"62","author":"Selcen","year":"2004","journal-title":"Neurology"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1002\/mus.21642","article-title":"Pre- and postsynaptic neuromuscular junction abnormalities in musk myasthenia","volume":"42","author":"Niks","year":"2010","journal-title":"Muscle Nerve"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1016\/j.expneurol.2012.01.025","article-title":"Passive and active immunization models of MuSK-Ab positive myasthenia: Electrophysiological evidence for pre and postsynaptic defects","volume":"234","author":"Viegas","year":"2012","journal-title":"Exp. Neurol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1016\/j.expneurol.2012.06.034","article-title":"Muscle specific kinase autoantibodies cause synaptic failure through progressive wastage of postsynaptic acetylcholine receptors","volume":"237","author":"Morsch","year":"2012","journal-title":"Exp. Neurol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"798","DOI":"10.1016\/j.ajpath.2011.10.031","article-title":"Antibodies against muscle-specific kinase impair both presynaptic and postsynaptic functions in a murine model of myasthenia gravis","volume":"180","author":"Mori","year":"2012","journal-title":"Am. J. Pathol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1439","DOI":"10.1001\/jama.1957.02980130015004","article-title":"Chronic thyroiditis and autoimmunization","volume":"164","author":"Witebsky","year":"1957","journal-title":"J. Am. Med Assoc."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1016\/0167-5699(93)90244-F","article-title":"Defining criteria for autoimmune diseases (Witebsky\u2019s postulates revisited)","volume":"14","author":"Rose","year":"1993","journal-title":"Immunol. Today"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1111\/nyas.13592","article-title":"Serological and experimental studies in different forms of myasthenia gravis","volume":"1413","author":"Vincent","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1111\/j.1749-6632.2012.06799.x","article-title":"Thymus pathology observed in the MGTX trial","volume":"1275","author":"Marx","year":"2012","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1056\/NEJMoa1602489","article-title":"Randomized Trial of Thymectomy in Myasthenia Gravis","volume":"375","author":"Wolfe","year":"2016","journal-title":"N. Engl. J. Med."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1002\/ana.20386","article-title":"Fewer thymic changes in MuSK antibody-positive than in MuSK antibody-negative MG","volume":"57","author":"Leite","year":"2005","journal-title":"Ann. Neurol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1002\/mus.26404","article-title":"Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis","volume":"59","author":"Clifford","year":"2019","journal-title":"Muscle Nerve"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1002\/mus.26439","article-title":"The evidence is stacked against thymectomy in MuSK myasthenia gravis","volume":"59","author":"Kushlaf","year":"2019","journal-title":"Muscle Nerve"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"931","DOI":"10.1016\/j.autrev.2013.03.004","article-title":"Diagnosis and therapy of myasthenia gravis with antibodies to muscle-specific kinase","volume":"12","author":"Evoli","year":"2013","journal-title":"Autoimmun. Rev."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1111\/j.1749-6632.2012.06781.x","article-title":"Management challenges in muscle-specific tyrosine kinase myasthenia gravis","volume":"1274","author":"Evoli","year":"2012","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1002\/mus.22006","article-title":"Anti-MuSK antibody myasthenia gravis: Clinical findings and response to treatment in two large cohorts","volume":"44","author":"Guptill","year":"2011","journal-title":"Muscle Nerve"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1099","DOI":"10.1136\/jnnp.2004.052415","article-title":"The features of myasthenia gravis with autoantibodies to MuSK","volume":"76","author":"Lavrnic","year":"2005","journal-title":"J. Neurol. Neurosurg Psychiatry"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1016\/j.jaut.2013.12.004","article-title":"A comprehensive analysis of the epidemiology and clinical characteristics of anti-LRP4 in myasthenia gravis","volume":"52","author":"Zisimopoulou","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_49","doi-asserted-by":"crossref","unstructured":"Tomschik, M., Koneczny, I., Sch\u00f6tta, A.-M., Scharer, S., Smajlhodzic, M., Rosenegger, P.F., Bl\u00fcthner, M., H\u00f6ftberger, R., Zimprich, F., and Stanek, G. (2019). Severe myasthenic manifestation of leptospirosis associated with a new sequence type of Leptospira interrogans acquired in Asia and diagnosed in Austria. Emerg. Infect. Dis.","DOI":"10.3201\/eid2505.181591"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.autrev.2018.07.011","article-title":"Characterization of the thymus in Lrp4 myasthenia gravis: Four cases","volume":"18","author":"Koneczny","year":"2019","journal-title":"Autoimmun. Rev."},{"key":"ref_51","unstructured":"Willis, T. (1672). De Anima Brutorum, Oxonii Theatro Sheldoniano."},{"key":"ref_52","first-page":"7","article-title":"On cerebritis, hysteria, and bulbar paralysis, as illustrative of arrest function of the cerebrospinal centers","volume":"22","author":"Wilks","year":"1877","journal-title":"Guy\u2019s Hosp. Rep."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1007\/BF02666475","article-title":"Zur casuistik der bulb\u00e4ren l\u00e4hmungen","volume":"9","author":"Erb","year":"1879","journal-title":"Arch. Psychiatr. Nervenkr."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1007\/BF01665294","article-title":"Ueber einen scheinbar heilbaren bulb\u00e4r paralytischen Symptomencomplex mit Betheiligung der Extremit\u00e4ten","volume":"4","author":"Goldflam","year":"1893","journal-title":"Dtsch. Z. Nervenheilkd."},{"key":"ref_55","first-page":"1","article-title":"Ueber Myasthenia gravis pseudoparalytica","volume":"32","author":"Jolly","year":"1895","journal-title":"Berl. Klin. Wochenschr."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1007\/BF01738910","article-title":"\u00dcber humorale \u00dcbertragbarkeit der Herznervenwirkung","volume":"189","author":"Loewi","year":"1922","journal-title":"Pfl\u00fcgers Arch. Ges Physiol."},{"key":"ref_57","first-page":"39P","article-title":"Chemical transmission at motor nerve endings in voluntary muscle","volume":"81","author":"Dale","year":"1934","journal-title":"J. Physiol. (Lond.)"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1113\/jphysiol.1936.sp003371","article-title":"Release of acetylcholine at voluntary motor nerve endings","volume":"86","author":"Dale","year":"1936","journal-title":"J. Physiol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/S0733-8619(18)30094-X","article-title":"The history of myasthenia gravis","volume":"12","author":"Pascuzzi","year":"1994","journal-title":"Neurol. Clin."},{"key":"ref_60","first-page":"539","article-title":"Mise en \u00e9vidence d\u2019une malformation caract\u00e9ristique de la jonction neuromusculaire dans la myasth\u00e9nie","volume":"59","author":"Coers","year":"1959","journal-title":"Acta Neurol. Psychiatr. Belg."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1038\/86280","article-title":"Autoimmunity versus horror autotoxicus: The struggle for recognition","volume":"2","author":"Silverstein","year":"2001","journal-title":"Nat. Immunol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1177\/003693306000501001","article-title":"Myasthenia gravis. A new hypothesis","volume":"4","author":"Simpson","year":"1960","journal-title":"Scot. Med. J."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"177","DOI":"10.3181\/00379727-105-26050","article-title":"Changes in serum complement activity in patients will myasthenia gravis","volume":"105","author":"Nastuk","year":"1960","journal-title":"Proc. Soc. Exp. Biol. Med."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1126\/science.180.4088.871","article-title":"Autoimmune response to acetylcholine receptor","volume":"180","author":"Patrick","year":"1973","journal-title":"Science"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1054","DOI":"10.1212\/WNL.26.11.1054","article-title":"Antibody to acetylcholine receptor in myasthenia gravis. Prevalence, clinical correlates, and diagnostic value","volume":"26","author":"Lindstrom","year":"1976","journal-title":"Neurology"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1126\/science.1179220","article-title":"Myasthenia gravis: Passive transfer from man to mouse","volume":"190","author":"Toyka","year":"1975","journal-title":"Science"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1073\/pnas.67.3.1241","article-title":"Use of a snake venom toxin to characterize the cholinergic receptor protein","volume":"67","author":"Changeux","year":"1970","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1111\/j.1476-5381.1966.tb01883.x","article-title":"Electrophysiological study of neuromuscular blocking with cobra neurotoxin","volume":"28","author":"Chang","year":"1966","journal-title":"Br. J. Pharm. Chemother."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1038\/229554a0","article-title":"Isolation of the cholinergic receptor protein of\u2019 Torpedo electric tissue","volume":"229","author":"Miledi","year":"1971","journal-title":"Nature"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"3334","DOI":"10.1073\/pnas.70.12.3334","article-title":"Studies on purified eel acetylcholine receptor and anti-acetylcholine receptor antibody","volume":"70","author":"Patrick","year":"1973","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1126\/science.182.4109.293","article-title":"Neuromuscular junction in myasthenia gravis: Decreased acetylcholine receptors","volume":"182","author":"Fambrough","year":"1973","journal-title":"Science"},{"key":"ref_72","first-page":"267","article-title":"Immune complexes (IgG and C3) at the motor end-plate in myasthenia gravis: Ultrastructural and light microscopic localization and electrophysiologic correlations","volume":"52","author":"Engel","year":"1977","journal-title":"Mayo. Clin. Proc."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1097\/00005072-198003000-00005","article-title":"Ultrastructural localization of the terminal and lytic ninth complement component (C9) at the motor end-plate in myasthenia gravis","volume":"39","author":"Sahashi","year":"1980","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1038\/85520","article-title":"Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies","volume":"7","author":"Hoch","year":"2001","journal-title":"Nat. Med."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1001\/archneurol.2011.2393","article-title":"Autoantibodies to lipoprotein-related protein 4 in patients with double-seronegative myasthenia gravis","volume":"69","author":"Zhang","year":"2012","journal-title":"Arch. Neurol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1002\/ana.22312","article-title":"Autoantibodies to low-density lipoprotein receptor-related protein 4 in myasthenia gravis","volume":"69","author":"Higuchi","year":"2011","journal-title":"Ann. Neurol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1007\/s00415-011-6194-7","article-title":"Anti-LRP4 autoantibodies in AChR- and MuSK-antibody-negative myasthenia gravis","volume":"259","author":"Pevzner","year":"2012","journal-title":"J. Neurol."},{"key":"ref_78","doi-asserted-by":"crossref","unstructured":"Zhang, B., Shen, C., Bealmear, B., Ragheb, S., Xiong, W.C., Lewis, R.A., Lisak, R.P., and Mei, L. (2014). Autoantibodies to agrin in myasthenia gravis patients. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0091816"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"1976","DOI":"10.1212\/WNL.0000000000000478","article-title":"Anti-agrin autoantibodies in myasthenia gravis","volume":"82","author":"Gasperi","year":"2014","journal-title":"Neurology"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/j.jns.2014.12.015","article-title":"Collagen Q--a potential target for autoantibodies in myasthenia gravis","volume":"348","author":"Belaya","year":"2015","journal-title":"J. Neurol. Sci."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/0022-510X(88)90170-0","article-title":"Passive transfer of myasthenia gravis by immunoglobulins: Lack of correlation between AChR with antibody bound, acetylcholine receptor loss and transmission defect","volume":"84","author":"Mossman","year":"1988","journal-title":"J. Neurol. Sci."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1393","DOI":"10.1002\/mus.880171208","article-title":"Passive transfer of seronegative myasthenia gravis to mice","volume":"17","author":"Burges","year":"1994","journal-title":"Muscle Nerve"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"3217","DOI":"10.1113\/jphysiol.2010.190298","article-title":"Patient autoantibodies deplete postsynaptic muscle-specific kinase leading to disassembly of the ACh receptor scaffold and myasthenia gravis in mice","volume":"588","author":"Cole","year":"2010","journal-title":"J. Physiol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1093\/brain\/aws025","article-title":"Muscle-specific kinase myasthenia gravis IgG4 autoantibodies cause severe neuromuscular junction dysfunction in mice","volume":"135","author":"Klooster","year":"2012","journal-title":"Brain J. Neurol."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1016\/j.expneurol.2017.08.006","article-title":"Immunization of mice with LRP4 induces myasthenia similar to MuSK-associated myasthenia gravis","volume":"297","author":"Mori","year":"2017","journal-title":"Exp. Neurol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"5190","DOI":"10.1172\/JCI66039","article-title":"Antibodies against low-density lipoprotein receptor-related protein 4 induce myasthenia gravis","volume":"123","author":"Shen","year":"2013","journal-title":"J. Clin. Investig."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1080\/08820139.2017.1299754","article-title":"Immunization with Recombinantly Expressed LRP4 Induces Experimental Autoimmune Myasthenia Gravis in C57BL\/6 Mice","volume":"46","author":"Ulusoy","year":"2017","journal-title":"Immunol. Investig."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.neuroscience.2018.01.015","article-title":"Induction of Anti-agrin Antibodies Causes Myasthenia Gravis in Mice","volume":"373","author":"Yan","year":"2018","journal-title":"Neuroscience"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1111\/j.1365-2249.1990.tb05440.x","article-title":"Patients with myasthenia gravis and thymoma have in their sera IgG autoantibodies against titin","volume":"82","author":"Aarli","year":"1990","journal-title":"Clin. Exp. Immunol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/j.jneuroim.2005.08.017","article-title":"Novel autoantibodies to a voltage-gated potassium channel Kv1.4 in a severe form of myasthenia gravis","volume":"170","author":"Suzuki","year":"2005","journal-title":"J. Neuroimmunol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1002\/ana.410320419","article-title":"Ryanodine receptor autoantibodies in myasthenia gravis patients with a thymoma","volume":"32","author":"Mygland","year":"1992","journal-title":"Ann. Neurol."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1016\/j.autrev.2014.08.039","article-title":"Cortactin autoantibodies in myasthenia gravis","volume":"13","author":"Gallardo","year":"2014","journal-title":"Autoimmun. Rev."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"1099","DOI":"10.1001\/jamaneurol.2016.2032","article-title":"Clinical Characteristics of Patients With Double-Seronegative Myasthenia Gravis and Antibodies to Cortactin","volume":"73","author":"Gallardo","year":"2016","journal-title":"JAMA Neurol."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1111\/nyas.13502","article-title":"Diagnostic utility of cortactin antibodies in myasthenia gravis","volume":"1412","author":"Illa","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1016\/j.jneuroim.2016.01.018","article-title":"Titin antibodies in \u201cseronegative\u201d myasthenia gravis--A new role for an old antigen","volume":"292","author":"Stergiou","year":"2016","journal-title":"J. Neuroimmunol."},{"key":"ref_96","first-page":"740583","article-title":"Three types of striational antibodies in myasthenia gravis","volume":"2011","author":"Suzuki","year":"2011","journal-title":"Autoimmune Dis."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1155\/1998\/49484","article-title":"Paraneoplastic autoimmunity in thymus tumors","volume":"6","author":"Marx","year":"1998","journal-title":"Dev. Immunol."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1111\/j.1749-6632.1998.tb10971.x","article-title":"Muscle striation antibodies in myasthenia gravis. Diagnostic and functional significance","volume":"841","author":"Aarli","year":"1998","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1038\/nri916","article-title":"Unravelling the pathogenesis of myasthenia gravis","volume":"2","author":"Vincent","year":"2002","journal-title":"Nat. Rev. Immunol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1113\/jphysiol.1955.sp005297","article-title":"On the localization of acetylcholine receptors","volume":"128","author":"Katz","year":"1955","journal-title":"J. Physiol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1016\/0006-8993(73)90158-3","article-title":"Ultrastructure of isolated membranes of Torpedo electric tissue","volume":"57","author":"Nickel","year":"1973","journal-title":"Brain Res."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1038\/336247a0","article-title":"Ion channel of acetylcholine receptor reconstructed from images of postsynaptic membranes","volume":"336","author":"Toyoshima","year":"1988","journal-title":"Nature"},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1016\/0959-4388(93)90121-E","article-title":"Structure of nicotinic acetylcholine receptors","volume":"3","author":"Karlin","year":"1993","journal-title":"Curr. Opin. Neurobiol."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1002\/(SICI)1097-4598(200004)23:4<453::AID-MUS3>3.0.CO;2-O","article-title":"Acetylcholine receptors and myasthenia","volume":"23","author":"Lindstrom","year":"2000","journal-title":"Muscle Nerve"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1101\/SQB.1983.048.01.009","article-title":"Molecular cloning of the acetylcholine receptor","volume":"48","author":"Patrick","year":"1983","journal-title":"Cold Spring Harb. Symp. Quant Biol."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1111\/j.1749-6632.1993.tb22882.x","article-title":"cDNA and genomic clones encoding the human muscle acetylcholine receptor","volume":"681","author":"Beeson","year":"1993","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1006\/jmbi.1999.2721","article-title":"Nicotinic acetylcholine receptor at 4.6 A resolution: Transverse tunnels in the channel wall","volume":"288","author":"Miyazawa","year":"1999","journal-title":"J. Mol. Biol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1083\/jcb.153.1.1","article-title":"Agrin-induced phosphorylation of the acetylcholine receptor regulates cytoskeletal anchoring and clustering","volume":"153","author":"Borges","year":"2001","journal-title":"J. Cell Biol."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"4167","DOI":"10.1242\/dev.010702","article-title":"Synaptic differentiation is defective in mice lacking acetylcholine receptor beta-subunit tyrosine phosphorylation","volume":"134","author":"Friese","year":"2007","journal-title":"Development"},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1016\/j.jmb.2012.07.010","article-title":"Gating movement of acetylcholine receptor caught by plunge-freezing","volume":"422","author":"Unwin","year":"2012","journal-title":"J. Mol. Biol."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1038\/nature01748","article-title":"Structure and gating mechanism of the acetylcholine receptor pore","volume":"423","author":"Miyazawa","year":"2003","journal-title":"Nature"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1016\/j.jmb.2004.12.031","article-title":"Refined structure of the nicotinic acetylcholine receptor at 4A resolution","volume":"346","author":"Unwin","year":"2005","journal-title":"J. Mol. Biol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1083\/jcb.105.1.9","article-title":"Location of subunits within the acetylcholine receptor by electron image analysis of tubular crystals from Torpedo marmorata","volume":"105","author":"Kubalek","year":"1987","journal-title":"J. Cell Biol."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/0014-5793(90)80286-R","article-title":"Determination of amino acids critical to the main immunogenic region of intact acetylcholine receptors by in vitro mutagenesis","volume":"267","author":"Saedi","year":"1990","journal-title":"FEBS Lett."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1073\/pnas.77.2.755","article-title":"Monoclonal antibodies used to probe acetylcholine receptor structure: Localization of the main immunogenic region and detection of similarities between subunits","volume":"77","author":"Tzartos","year":"1980","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1083\/jcb.82.2.412","article-title":"Acetylcholine receptors in regenerating muscle accumulate at original synaptic sites in the absence of the nerve","volume":"82","author":"Burden","year":"1979","journal-title":"J. Cell Biol."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"3073","DOI":"10.1073\/pnas.74.7.3073","article-title":"Reinnervation of original synaptic sites on muscle fiber basement membrane after disruption of the muscle cells","volume":"74","author":"Marshall","year":"1977","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1083\/jcb.78.1.176","article-title":"Reinnervation of muscle fiber basal lamina after removal of myofibers. Differentiation of regenerating axons at original synaptic sites","volume":"78","author":"Sanes","year":"1978","journal-title":"J. Cell Biol."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1083\/jcb.99.2.615","article-title":"Components of Torpedo electric organ and muscle that cause aggregation of acetylcholine receptors on cultured muscle cells","volume":"99","author":"Godfrey","year":"1984","journal-title":"J. Cell Biol."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"2471","DOI":"10.1083\/jcb.105.6.2471","article-title":"Identification of agrin, a synaptic organizing protein from Torpedo electric organ","volume":"105","author":"Nitkin","year":"1987","journal-title":"J. Cell Biol."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1038\/nrm1074","article-title":"New insights into the roles of agrin","volume":"4","author":"Bezakova","year":"2003","journal-title":"Nat. Rev."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/S0896-6273(00)80751-5","article-title":"Alternatively spliced isoforms of nerve- and muscle-derived agrin: Their roles at the neuromuscular junction","volume":"23","author":"Burgess","year":"1999","journal-title":"Neuron"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"2814","DOI":"10.1002\/j.1460-2075.1994.tb06575.x","article-title":"Structural domains of agrin required for clustering of nicotinic acetylcholine receptors","volume":"13","author":"Hoch","year":"1994","journal-title":"EMBO J."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1016\/j.str.2004.02.001","article-title":"Modulation of agrin function by alternative splicing and Ca2+ binding","volume":"12","author":"Stetefeld","year":"2004","journal-title":"Structure"},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1101\/gad.180885.111","article-title":"Structural basis of agrin-LRP4-MuSK signaling","volume":"26","author":"Zong","year":"2012","journal-title":"Genes Dev."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/S0092-8674(00)81252-0","article-title":"Agrin acts via a MuSK receptor complex","volume":"85","author":"Glass","year":"1996","journal-title":"Cell"},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"4993","DOI":"10.1242\/dev.02696","article-title":"LDL-receptor-related protein 4 is crucial for formation of the neuromuscular junction","volume":"133","author":"Weatherbee","year":"2006","journal-title":"Development"},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"40624","DOI":"10.1074\/jbc.M111.279307","article-title":"Agrin binds to the N-terminal region of Lrp4 protein and stimulates association between Lrp4 and the first immunoglobulin-like domain in muscle-specific kinase (MuSK)","volume":"286","author":"Zhang","year":"2011","journal-title":"J. Biol. Chem."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"2895","DOI":"10.1073\/pnas.90.7.2895","article-title":"Muscle-specific trk-related receptor with a kringle domain defines a distinct class of receptor tyrosine kinases","volume":"90","author":"Jennings","year":"1993","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1016\/0896-6273(95)90146-9","article-title":"Receptor tyrosine kinase specific for the skeletal muscle lineage: Expression in embryonic muscle, at the neuromuscular junction, and after injury","volume":"15","author":"Valenzuela","year":"1995","journal-title":"Neuron"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"6184","DOI":"10.1038\/ncomms7184","article-title":"An oncogenic role of Agrin in regulating focal adhesion integrity in hepatocellular carcinoma","volume":"6","author":"Chakraborty","year":"2015","journal-title":"Nat. Commun."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1046\/j.1460-9568.1999.00443.x","article-title":"Xenopus muscle-specific kinase: Molecular cloning and prominent expression in neural tissues during early embryonic development","volume":"11","author":"Fu","year":"1999","journal-title":"Eur. J. Neurosci."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"7919","DOI":"10.1523\/JNEUROSCI.1674-06.2006","article-title":"MuSK expressed in the brain mediates cholinergic responses, synaptic plasticity, and memory formation","volume":"26","author":"Tsokas","year":"2006","journal-title":"J. Neurosci."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1006\/mcne.2000.0892","article-title":"Cloning and characterization of muscle-specific kinase in chicken","volume":"16","author":"Ip","year":"2000","journal-title":"Mol. Cell Neurosci."},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1016\/j.jmb.2006.09.019","article-title":"Crystal structure of the agrin-responsive immunoglobulin-like domains 1 and 2 of the receptor tyrosine kinase MuSK","volume":"364","author":"Stiegler","year":"2006","journal-title":"J. Mol. Biol."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"1133","DOI":"10.1083\/jcb.146.5.1133","article-title":"Distinct domains of MuSK mediate its abilities to induce and to associate with postsynaptic specializations","volume":"146","author":"Zhou","year":"1999","journal-title":"J. Cell Biol."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1186\/1756-6606-5-7","article-title":"Wnt proteins regulate acetylcholine receptor clustering in muscle cells","volume":"5","author":"Zhang","year":"2012","journal-title":"Mol. Brain"},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1242\/dev.071555","article-title":"Initiation of synapse formation by Wnt-induced MuSK endocytosis","volume":"139","author":"Gordon","year":"2012","journal-title":"Development"},{"key":"ref_139","doi-asserted-by":"crossref","unstructured":"Strochlic, L., Falk, J., Goillot, E., Sigoillot, S., Bourgeois, F., Delers, P., Rouviere, J., Swain, A., Castellani, V., and Schaeffer, L. (2012). Wnt4 participates in the formation of vertebrate neuromuscular junction. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0029976"},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"4926","DOI":"10.1523\/JNEUROSCI.3381-14.2015","article-title":"MuSK frizzled-like domain is critical for mammalian neuromuscular junction formation and maintenance","volume":"35","author":"Messeant","year":"2015","journal-title":"J. Neurosci."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"1058","DOI":"10.1101\/gad.279745.116","article-title":"Diverging roles for Lrp4 and Wnt signaling in neuromuscular synapse development during evolution","volume":"30","author":"Remedio","year":"2016","journal-title":"Genes Dev."},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"1187","DOI":"10.1016\/S0969-2126(02)00814-6","article-title":"Crystal structure of the MuSK tyrosine kinase: Insights into receptor autoregulation","volume":"10","author":"Till","year":"2002","journal-title":"Structure"},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"8848","DOI":"10.1073\/pnas.94.16.8848","article-title":"Kinase domain of the muscle-specific receptor tyrosine kinase (MuSK) is sufficient for phosphorylation but not clustering of acetylcholine receptors: Required role for the MuSK ectodomain?","volume":"94","author":"Glass","year":"1997","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/emboj\/19.1.67","article-title":"The juxtamembrane region of MuSK has a critical role in agrin-mediated signaling","volume":"19","author":"Herbst","year":"2000","journal-title":"EMBO J."},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"1802","DOI":"10.1126\/science.1127142","article-title":"The muscle protein Dok-7 is essential for neuromuscular synaptogenesis","volume":"312","author":"Okada","year":"2006","journal-title":"Science"},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"4585","DOI":"10.1073\/pnas.080061997","article-title":"The in vitro and in vivo phosphotyrosine map of activated MuSK","volume":"97","author":"Watty","year":"2000","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/S0009-2797(99)00040-X","article-title":"Acetylcholinesterase in the neuromuscular junction","volume":"119\u2013120","author":"Gaspersic","year":"1999","journal-title":"Chem. Biol. Interact"},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.1002\/j.1460-2075.1991.tb08070.x","article-title":"Primary structure of a collagenic tail peptide of Torpedo acetylcholinesterase: Co-expression with catalytic subunit induces the production of collagen-tailed forms in transfected cells","volume":"10","author":"Krejci","year":"1991","journal-title":"EMBO J."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"1349","DOI":"10.1083\/jcb.144.6.1349","article-title":"Genetic analysis of collagen Q: Roles in acetylcholinesterase and butyrylcholinesterase assembly and in synaptic structure and function","volume":"144","author":"Feng","year":"1999","journal-title":"J. Cell Biol."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1038\/nrn1101","article-title":"Sleuthing molecular targets for neurological diseases at the neuromuscular junction","volume":"4","author":"Engel","year":"2003","journal-title":"Nat. Rev. Neurosci."},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1083\/jcb.200307164","article-title":"MuSK is required for anchoring acetylcholinesterase at the neuromuscular junction","volume":"165","author":"Cartaud","year":"2004","journal-title":"J. Cell Biol."},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1016\/0955-0674(92)90113-Q","article-title":"Agrin isoforms and their role in synaptogenesis","volume":"4","author":"McMahan","year":"1992","journal-title":"Curr. Opin. Cell Biol."},{"key":"ref_153","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1016\/j.cell.2008.10.002","article-title":"Lrp4 is a receptor for Agrin and forms a complex with MuSK","volume":"135","author":"Kim","year":"2008","journal-title":"Cell"},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/j.neuron.2008.10.006","article-title":"LRP4 serves as a coreceptor of agrin","volume":"60","author":"Zhang","year":"2008","journal-title":"Neuron"},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"ra7","DOI":"10.1126\/scisignal.2000113","article-title":"Dok-7 activates the muscle receptor kinase MuSK and shapes synapse formation","volume":"2","author":"Inoue","year":"2009","journal-title":"Sci. Signal."},{"key":"ref_156","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1016\/j.molcel.2010.06.007","article-title":"The cytoplasmic adaptor protein Dok7 activates the receptor tyrosine kinase MuSK via dimerization","volume":"39","author":"Bergamin","year":"2010","journal-title":"Mol. Cell"},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1038\/35097557","article-title":"Induction, assembly, maturation and maintenance of a postsynaptic apparatus","volume":"2","author":"Sanes","year":"2001","journal-title":"Nat. Rev. Neurosci."},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1016\/S0092-8674(00)81251-9","article-title":"The receptor tyrosine kinase MuSK is required for neuromuscular junction formation in vivo","volume":"85","author":"DeChiara","year":"1996","journal-title":"Cell"},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1016\/S0092-8674(00)81253-2","article-title":"Defective neuromuscular synaptogenesis in agrin-deficient mutant mice","volume":"85","author":"Gautam","year":"1996","journal-title":"Cell"},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1038\/35074025","article-title":"Distinct roles of nerve and muscle in postsynaptic differentiation of the neuromuscular synapse","volume":"410","author":"Lin","year":"2001","journal-title":"Nature"},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1016\/S0896-6273(01)00287-2","article-title":"Patterning of muscle acetylcholine receptor gene expression in the absence of motor innervation","volume":"30","author":"Yang","year":"2001","journal-title":"Neuron"},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1126\/science.287.5450.131","article-title":"DNA topoisomerase IIbeta and neural development","volume":"287","author":"Yang","year":"2000","journal-title":"Science"},{"key":"ref_163","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1016\/j.mcn.2004.10.005","article-title":"Tyrosine phosphatase regulation of MuSK-dependent acetylcholine receptor clustering","volume":"28","author":"Madhavan","year":"2005","journal-title":"Mol. Cell Neurosci."},{"key":"ref_164","doi-asserted-by":"crossref","first-page":"13892","DOI":"10.1523\/JNEUROSCI.1733-14.2014","article-title":"LRP4 is critical for neuromuscular junction maintenance","volume":"34","author":"Barik","year":"2014","journal-title":"J. Neurosci."},{"key":"ref_165","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1016\/j.mcn.2005.10.020","article-title":"Synapse disassembly and formation of new synapses in postnatal muscle upon conditional inactivation of MuSK","volume":"31","author":"Hesser","year":"2006","journal-title":"Mol. Cell Neurosci."},{"key":"ref_166","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1038\/sj.embor.7400065","article-title":"Inhibition of synapse assembly in mammalian muscle in vivo by RNA interference","volume":"5","author":"Kong","year":"2004","journal-title":"EMBO Rep."},{"key":"ref_167","doi-asserted-by":"crossref","unstructured":"Samuel, M.A., Valdez, G., Tapia, J.C., Lichtman, J.W., and Sanes, J.R. (2012). Agrin and synaptic laminin are required to maintain adult neuromuscular junctions. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0046663"},{"key":"ref_168","doi-asserted-by":"crossref","first-page":"3944","DOI":"10.1242\/jcs.072090","article-title":"Specific proteolytic cleavage of agrin regulates maturation of the neuromuscular junction","volume":"123","author":"Bolliger","year":"2010","journal-title":"J. Cell Sci."},{"key":"ref_169","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1016\/j.mcn.2011.12.007","article-title":"The formation of complex acetylcholine receptor clusters requires MuSK kinase activity and structural information from the MuSK extracellular domain","volume":"49","author":"Mazhar","year":"2012","journal-title":"Mol. Cell Neurosci."},{"key":"ref_170","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1097\/WCO.0000000000000370","article-title":"Congenital myasthenic syndromes: Recent advances","volume":"29","author":"Beeson","year":"2016","journal-title":"Curr. Opin. Neurol."},{"key":"ref_171","doi-asserted-by":"crossref","unstructured":"Rodriguez Cruz, P.M., Palace, J., and Beeson, D. (2018). The Neuromuscular Junction and Wide Heterogeneity of Congenital Myasthenic Syndromes. Int. J. Mol. Sci., 19.","DOI":"10.3390\/ijms19061677"},{"key":"ref_172","first-page":"94","article-title":"Sarcopenia: Prevalence, mechanisms, and functional consequences","volume":"37","author":"Berger","year":"2010","journal-title":"Interdiscip. Top. Gerontol."},{"key":"ref_173","doi-asserted-by":"crossref","first-page":"14863","DOI":"10.1073\/pnas.1002220107","article-title":"Attenuation of age-related changes in mouse neuromuscular synapses by caloric restriction and exercise","volume":"107","author":"Valdez","year":"2010","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_174","doi-asserted-by":"crossref","unstructured":"Chai, R.J., Vukovic, J., Dunlop, S., Grounds, M.D., and Shavlakadze, T. (2011). Striking denervation of neuromuscular junctions without lumbar motoneuron loss in geriatric mouse muscle. PLoS ONE, 6.","DOI":"10.1371\/journal.pone.0028090"},{"key":"ref_175","doi-asserted-by":"crossref","first-page":"13191","DOI":"10.1523\/JNEUROSCI.2953-11.2011","article-title":"Nerve terminal growth remodels neuromuscular synapses in mice following regeneration of the postsynaptic muscle fiber","volume":"31","author":"Li","year":"2011","journal-title":"J. Neurosci."},{"key":"ref_176","doi-asserted-by":"crossref","first-page":"24849","DOI":"10.1038\/srep24849","article-title":"Age-related fragmentation of the motor endplate is not associated with impaired neuromuscular transmission in the mouse diaphragm","volume":"6","author":"Willadt","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_177","doi-asserted-by":"crossref","first-page":"4378","DOI":"10.1096\/fj.11-191262","article-title":"Destabilization of the neuromuscular junction by proteolytic cleavage of agrin results in precocious sarcopenia","volume":"25","author":"Butikofer","year":"2011","journal-title":"FASEB J."},{"key":"ref_178","first-page":"82","article-title":"Acetylcholine receptor antibody in myasthenia gravis: Predominance of IgG subclasses 1 and 3","volume":"67","author":"Rodgaard","year":"1987","journal-title":"Clin. Exp. Immunol."},{"key":"ref_179","first-page":"257","article-title":"Acetylcholine receptor antibody characteristics in myasthenia gravis. I. Patients with generalized myasthenia or disease restricted to ocular muscles","volume":"49","author":"Vincent","year":"1982","journal-title":"Clin. Exp. Immunol."},{"key":"ref_180","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/0165-5728(81)90015-1","article-title":"Binding properties and subclass distribution of anti-acetylcholine receptor antibodies in myasthenia gravis","volume":"1","author":"Lefvert","year":"1981","journal-title":"J. Neuroimmunol."},{"key":"ref_181","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1002\/j.1460-2075.1983.tb01434.x","article-title":"High affinity binding of alpha-bungarotoxin to the purified alpha-subunit and to its 27,000-dalton proteolytic peptide from Torpedo marmorata acetylcholine receptor. Requirement for sodium dodecyl sulfate","volume":"2","author":"Tzartos","year":"1983","journal-title":"EMBO J."},{"key":"ref_182","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/j.1749-6632.1981.tb33721.x","article-title":"Structure and function of the acetylcholine receptor molecule studied using monoclonal antibodies","volume":"377","author":"Lindstrom","year":"1981","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_183","first-page":"419","article-title":"Antibodies to acetylcholine receptors as probes of receptor structure and pathological agents in myasthenia gravis","volume":"63","author":"Lindstrom","year":"1981","journal-title":"Prog. Clin. Biol. Res."},{"key":"ref_184","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1136\/jnnp-2012-302705","article-title":"Antibodies against the main immunogenic region of the acetylcholine receptor correlate with disease severity in myasthenia gravis","volume":"83","author":"Masuda","year":"2012","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_185","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1111\/nyas.13522","article-title":"Myasthenia gravis: The role of complement at the neuromuscular junction","volume":"1412","author":"Howard","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_186","doi-asserted-by":"crossref","first-page":"1167","DOI":"10.1212\/WNL.43.6.1167","article-title":"Myasthenia gravis: Quantitative immunocytochemical analysis of inflammatory cells and detection of complement membrane attack complex at the end-plate in 30 patients","volume":"43","author":"Nakano","year":"1993","journal-title":"Neurology"},{"key":"ref_187","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1111\/j.1749-6632.1987.tb51301.x","article-title":"The membrane attack complex of complement at the endplate in myasthenia gravis","volume":"505","author":"Engel","year":"1987","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_188","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/0303-8467(93)90103-N","article-title":"Soluble terminal complement components in human myasthenia gravis","volume":"95","author":"Barohn","year":"1993","journal-title":"Clin Neurol Neurosurg"},{"key":"ref_189","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/j.jneuroim.2004.08.002","article-title":"The role of complement in myasthenia gravis: Serological evidence of complement consumption in vivo","volume":"158","author":"Romi","year":"2005","journal-title":"J. Neuroimmunol."},{"key":"ref_190","doi-asserted-by":"crossref","first-page":"10302","DOI":"10.1523\/JNEUROSCI.3408-04.2004","article-title":"Structural abnormalities at neuromuscular synapses lacking multiple syntrophin isoforms","volume":"24","author":"Adams","year":"2004","journal-title":"J. Neurosci."},{"key":"ref_191","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.cellsig.2008.06.020","article-title":"Clathrin-independent endocytosis: A unique platform for cell signaling and PM remodeling","volume":"21","author":"Donaldson","year":"2009","journal-title":"Cell Signal."},{"key":"ref_192","doi-asserted-by":"crossref","first-page":"1748","DOI":"10.1212\/WNL.35.12.1748","article-title":"Complement-dependent lysis of cultured rat myotubes by myasthenic immunoglobulins","volume":"35","author":"Ashizawa","year":"1985","journal-title":"Neurology"},{"key":"ref_193","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1016\/S0165-5728(96)00144-0","article-title":"Soluble complement receptor 1 (sCR1) protects against experimental autoimmune myasthenia gravis","volume":"71","author":"Piddlesden","year":"1996","journal-title":"J. Neuroimmunol."},{"key":"ref_194","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1016\/j.jaut.2013.01.003","article-title":"Complement C2 siRNA mediated therapy of myasthenia gravis in mice","volume":"42","author":"Huda","year":"2013","journal-title":"J. Autoimmun."},{"key":"ref_195","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1016\/j.omtm.2019.04.009","article-title":"Investigational RNAi Therapeutic Targeting C5 Is Efficacious in Pre-clinical Models of Myasthenia Gravis","volume":"13","author":"Kusner","year":"2019","journal-title":"Mol. Methods Clin. Dev."},{"key":"ref_196","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1002\/mus.24057","article-title":"Targeting therapy to the neuromuscular junction: Proof of concept","volume":"49","author":"Kusner","year":"2014","journal-title":"Muscle Nerve"},{"key":"ref_197","doi-asserted-by":"crossref","first-page":"2343","DOI":"10.4049\/jimmunol.134.4.2343","article-title":"Role of the main immunogenic region of acetylcholine receptor in myasthenia gravis. An Fab monoclonal antibody protects against antigenic modulation by human sera","volume":"134","author":"Tzartos","year":"1985","journal-title":"J. Immunol."},{"key":"ref_198","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1016\/0301-0082(85)90018-8","article-title":"Nicotinic acetylcholine receptors in vertebrate muscle: Properties, distribution and neural control","volume":"25","author":"Salpeter","year":"1985","journal-title":"Prog. Neurobiol."},{"key":"ref_199","doi-asserted-by":"crossref","first-page":"6320","DOI":"10.1016\/S0021-9258(18)50365-3","article-title":"Acetylcholine receptor degradation in adult rat diaphragms in organ culture and the effect of anti-acetylcholine receptor antibodies","volume":"254","author":"Merlie","year":"1979","journal-title":"J. Biol. Chem."},{"key":"ref_200","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1126\/science.850793","article-title":"Myasthenic immunoglobulin accelerates acetylcholine receptor degradation","volume":"196","author":"Kao","year":"1977","journal-title":"Science"},{"key":"ref_201","doi-asserted-by":"crossref","first-page":"1116","DOI":"10.1056\/NEJM197805182982004","article-title":"Myasthenic antibodies cross-link acetylcholine receptors to accelerate degradation","volume":"298","author":"Drachman","year":"1978","journal-title":"N. Engl. J. Med."},{"key":"ref_202","doi-asserted-by":"crossref","first-page":"3090","DOI":"10.1073\/pnas.74.7.3090","article-title":"Modulation of acetylcholine receptor by antibody against the receptor","volume":"74","author":"Heinemann","year":"1977","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_203","doi-asserted-by":"crossref","unstructured":"Lee, C.W., Zhang, H., Geng, L., and Peng, H.B. (2014). Crosslinking-induced endocytosis of acetylcholine receptors by quantum dots. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0090187"},{"key":"ref_204","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1111\/j.1749-6632.1993.tb22936.x","article-title":"Seronegative myasthenia gravis. Evidence for plasma factor(s) interfering with acetylcholine receptor function","volume":"681","author":"Vincent","year":"1993","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_205","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1111\/j.1749-6632.1987.tb51321.x","article-title":"Clinical correlations of antibodies that bind, block, or modulate human acetylcholine receptors in myasthenia gravis","volume":"505","author":"Howard","year":"1987","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_206","doi-asserted-by":"crossref","first-page":"4089","DOI":"10.1073\/pnas.80.13.4089","article-title":"Anti-acetylcholine receptor antibodies directed against the alpha-bungarotoxin binding site induce a unique form of experimental myasthenia","volume":"80","author":"Gomez","year":"1983","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_207","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/0165-5728(83)90022-X","article-title":"Acetylcholine receptor antibody characteristics in myasthenia gravis. Fractionation of alpha-bungarotoxin binding site antibodies and their relationship to IgG subclass","volume":"5","author":"Whiting","year":"1983","journal-title":"J. Neuroimmunol."},{"key":"ref_208","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1212\/WNL.43.5.983","article-title":"The \u2018embryonic\u2019 gamma subunit of the nicotinic acetylcholine receptor is expressed in adult extraocular muscle","volume":"43","author":"Horton","year":"1993","journal-title":"Neurology"},{"key":"ref_209","doi-asserted-by":"crossref","first-page":"1402","DOI":"10.1111\/ene.13788","article-title":"Maternal myasthenia gravis represents a risk for the child through autoantibody transfer, immunosuppressive therapy and genetic influence","volume":"25","author":"Gilhus","year":"2018","journal-title":"Eur. J. Neurol."},{"key":"ref_210","doi-asserted-by":"crossref","first-page":"580","DOI":"10.1002\/ana.20061","article-title":"Detection and characterization of MuSK antibodies in seronegative myasthenia gravis","volume":"55","author":"McConville","year":"2004","journal-title":"Ann. Neurol."},{"key":"ref_211","doi-asserted-by":"crossref","unstructured":"Koneczny, I., Cossins, J., Waters, P., Beeson, D., and Vincent, A. (2013). MuSK Myasthenia Gravis IgG4 Disrupts the Interaction of LRP4 with MuSK but Both IgG4 and IgG1-3 Can Disperse Preformed Agrin-Independent AChR Clusters. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0080695"},{"key":"ref_212","doi-asserted-by":"crossref","first-page":"97","DOI":"10.3389\/fimmu.2018.00097","article-title":"A New Classification System for IgG4 Autoantibodies","volume":"9","author":"Koneczny","year":"2018","journal-title":"Front. Immunol."},{"key":"ref_213","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1016\/j.jaut.2016.11.005","article-title":"IgG4 autoantibodies against muscle-specific kinase undergo Fab-arm exchange in myasthenia gravis patients","volume":"77","author":"Koneczny","year":"2017","journal-title":"J. Autoimmun."},{"key":"ref_214","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1016\/j.jneuroim.2015.12.016","article-title":"Longitudinal epitope mapping in MuSK myasthenia gravis: Implications for disease severity","volume":"291","author":"Huijbers","year":"2016","journal-title":"J. Neuroimmunol."},{"key":"ref_215","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/j.jneuroim.2012.09.001","article-title":"Antibodies against Wnt receptor of muscle-specific tyrosine kinase in myasthenia gravis","volume":"254","author":"Takamori","year":"2013","journal-title":"J. Neuroimmunol."},{"key":"ref_216","doi-asserted-by":"crossref","first-page":"13928","DOI":"10.1038\/srep13928","article-title":"Collagen Q and anti-MuSK autoantibody competitively suppress agrin\/LRP4\/MuSK signaling","volume":"5","author":"Otsuka","year":"2015","journal-title":"Sci. Rep."},{"key":"ref_217","doi-asserted-by":"crossref","unstructured":"Huijbers, M.G., Zhang, W., Klooster, R., Niks, E.H., Friese, M.B., Straasheijm, K.R., Thijssen, P.E., Vrolijk, H., Plomp, J.J., and Vogels, P. (2013). MuSK IgG4 autoantibodies cause myasthenia gravis by inhibiting binding between MuSK and Lrp4. Proc. Natl. Acad. Sci. USA.","DOI":"10.1073\/pnas.1313944110"},{"key":"ref_218","doi-asserted-by":"crossref","first-page":"6467","DOI":"10.1074\/jbc.273.11.6467","article-title":"Dimerization of the muscle-specific kinase induces tyrosine phosphorylation of acetylcholine receptors and their aggregation on the surface of myotubes","volume":"273","author":"Hopf","year":"1998","journal-title":"J. Biol. Chem."},{"key":"ref_219","doi-asserted-by":"crossref","first-page":"e547","DOI":"10.1212\/NXI.0000000000000547","article-title":"MuSK myasthenia gravis monoclonal antibodies: Valency dictates pathogenicity","volume":"6","author":"Huijbers","year":"2019","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_220","unstructured":"Koneczny, I. (2014). Potential Mechanisms in MuSK Myasthenia Gravis. [Ph.D. Dissertation, Oxford University]."},{"key":"ref_221","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1038\/nature11348","article-title":"Lrp4 is a retrograde signal for presynaptic differentiation at neuromuscular synapses","volume":"489","author":"Yumoto","year":"2012","journal-title":"Nature"},{"key":"ref_222","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1016\/j.neuron.2012.04.033","article-title":"Distinct roles of muscle and motoneuron LRP4 in neuromuscular junction formation","volume":"75","author":"Wu","year":"2012","journal-title":"Neuron"},{"key":"ref_223","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1038\/ni1076","article-title":"Receptor editing is the main mechanism of B cell tolerance toward membrane antigens","volume":"5","author":"Halverson","year":"2004","journal-title":"Nat. Immunol."},{"key":"ref_224","doi-asserted-by":"crossref","first-page":"632","DOI":"10.1016\/j.coi.2008.09.001","article-title":"B-cell tolerance checkpoints in health and autoimmunity","volume":"20","author":"Meffre","year":"2008","journal-title":"Curr. Opin. Immunol."},{"key":"ref_225","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1038\/nri.2017.19","article-title":"Mechanisms of central tolerance for B cells","volume":"17","author":"Nemazee","year":"2017","journal-title":"Nat. Rev. Immunol."},{"key":"ref_226","doi-asserted-by":"crossref","first-page":"1460","DOI":"10.4049\/jimmunol.1601415","article-title":"Dysregulation of B Cell Repertoire Formation in Myasthenia Gravis Patients Revealed through Deep Sequencing","volume":"198","author":"Stathopoulos","year":"2017","journal-title":"J. Immunol."},{"key":"ref_227","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1002\/acn3.311","article-title":"Compromised fidelity of B-cell tolerance checkpoints in AChR and MuSK myasthenia gravis","volume":"3","author":"Lee","year":"2016","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_228","doi-asserted-by":"crossref","first-page":"412","DOI":"10.3109\/08916934.2015.1031890","article-title":"Poly-autoimmunity in patients with myasthenia gravis: A single-center experience","volume":"48","author":"Evoli","year":"2015","journal-title":"Autoimmunity"},{"key":"ref_229","doi-asserted-by":"crossref","first-page":"34","DOI":"10.3389\/fneur.2017.00034","article-title":"B10 Cell Frequencies and Suppressive Capacity in Myasthenia Gravis Are Associated with Disease Severity","volume":"8","author":"Yi","year":"2017","journal-title":"Front. Neurol."},{"key":"ref_230","doi-asserted-by":"crossref","first-page":"e77","DOI":"10.1212\/NXI.0000000000000077","article-title":"Characterization of B cells in muscle-specific kinase antibody myasthenia gravis","volume":"2","author":"Guptill","year":"2015","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_231","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1172\/JCI86800","article-title":"Estrogen turns down \u201cthe AIRE\u201d","volume":"126","author":"Bakhru","year":"2016","journal-title":"J. Clin. Investig."},{"key":"ref_232","doi-asserted-by":"crossref","first-page":"1525","DOI":"10.1172\/JCI81894","article-title":"Estrogen-mediated downregulation of AIRE influences sexual dimorphism in autoimmune diseases","volume":"126","author":"Dragin","year":"2016","journal-title":"J. Clin. Investig."},{"key":"ref_233","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1016\/j.immuni.2014.08.007","article-title":"Distinct contributions of Aire and antigen-presenting-cell subsets to the generation of self-tolerance in the thymus","volume":"41","author":"Perry","year":"2014","journal-title":"Immunity"},{"key":"ref_234","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1038\/nri3667","article-title":"Positive and negative selection of the T cell repertoire: What thymocytes see (and don\u2019t see)","volume":"14","author":"Klein","year":"2014","journal-title":"Nat. Rev. Immunol."},{"key":"ref_235","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1016\/j.autrev.2013.03.007","article-title":"The different roles of the thymus in the pathogenesis of the various myasthenia gravis subtypes","volume":"12","author":"Marx","year":"2013","journal-title":"Autoimmun. Rev."},{"key":"ref_236","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1002\/path.2141","article-title":"Deficiency of the autoimmune regulator AIRE in thymomas is insufficient to elicit autoimmune polyendocrinopathy syndrome type I (APS-I)","volume":"211","author":"Strobel","year":"2007","journal-title":"J. Pathol."},{"key":"ref_237","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.imlet.2014.04.008","article-title":"Autoimmune regulator expression in thymomas with or without autoimmune disease","volume":"161","author":"Liu","year":"2014","journal-title":"Immunol. Lett."},{"key":"ref_238","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1111\/nyas.13529","article-title":"AIRE: A missing link to explain female susceptibility to autoimmune diseases","volume":"1412","author":"Panse","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_239","first-page":"447","article-title":"Myasthenic and nonmyasthenic thymoma. An expansion of a minor cortical epithelial cell subset?","volume":"127","author":"Willcox","year":"1987","journal-title":"Am. J. Pathol."},{"key":"ref_240","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1002\/ana.20340","article-title":"Selective loss of regulatory T cells in thymomas","volume":"56","author":"Strobel","year":"2004","journal-title":"Ann. Neurol."},{"key":"ref_241","doi-asserted-by":"crossref","first-page":"413","DOI":"10.3109\/08916930903555935","article-title":"Thymoma and paraneoplastic myasthenia gravis","volume":"43","author":"Marx","year":"2010","journal-title":"Autoimmunity"},{"key":"ref_242","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/j.jneuroim.2013.09.006","article-title":"Disturbed B cell subpopulations and increased plasma cells in myasthenia gravis patients","volume":"264","author":"Kohler","year":"2013","journal-title":"J. Neuroimmunol."},{"key":"ref_243","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1097\/WCO.0b013e328359da15","article-title":"Paraneoplastic neurological syndromes","volume":"25","author":"Graus","year":"2012","journal-title":"Curr. Opin. Neurol."},{"key":"ref_244","doi-asserted-by":"crossref","first-page":"3752","DOI":"10.4049\/jimmunol.157.8.3752","article-title":"Expression of acetylcholine receptor genes in human thymic epithelial cells: Implications for myasthenia gravis","volume":"157","author":"Wakkach","year":"1996","journal-title":"J. Immunol."},{"key":"ref_245","doi-asserted-by":"crossref","first-page":"1140","DOI":"10.1172\/JCI112069","article-title":"Thymoma epithelial cells secrete thymic hormone but do not express class II antigens of the major histocompatibility complex","volume":"76","author":"Savino","year":"1985","journal-title":"J. Clin. Investig."},{"key":"ref_246","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1196\/annals.1405.018","article-title":"Common cellular and diverse genetic basis of thymoma-associated myasthenia gravis: Role of MHC class II and AIRE genes and genetic polymorphisms","volume":"1132","author":"Strobel","year":"2008","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_247","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.jaut.2013.12.011","article-title":"Myasthenia gravis: A comprehensive review of immune dysregulation and etiological mechanisms","volume":"52","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_248","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/S0165-5728(02)00038-3","article-title":"Thymic myoid cells and germinal center formation in myasthenia gravis; possible roles in pathogenesis","volume":"125","author":"Roxanis","year":"2002","journal-title":"J. Neuroimmunol."},{"key":"ref_249","doi-asserted-by":"crossref","first-page":"893","DOI":"10.2353\/ajpath.2007.070240","article-title":"Myasthenia gravis thymus: Complement vulnerability of epithelial and myoid cells, complement attack on them, and correlations with autoantibody status","volume":"171","author":"Leite","year":"2007","journal-title":"Am. J. Pathol."},{"key":"ref_250","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1002\/ana.410220205","article-title":"Acetylcholine receptors in human thymic myoid cells in situ: An immunohistological study","volume":"22","author":"Schluep","year":"1987","journal-title":"Ann. Neurol."},{"key":"ref_251","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1126\/science.831257","article-title":"Thymic muscle cells bear acetylcholine receptors: Possible relation to myasthenia gravis","volume":"195","author":"Kao","year":"1977","journal-title":"Science"},{"key":"ref_252","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1007\/s12016-016-8558-3","article-title":"Thymic Germinal Centers and Corticosteroids in Myasthenia Gravis: An Immunopathological Study in 1035 Cases and a Critical Review","volume":"52","author":"Truffault","year":"2017","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_253","doi-asserted-by":"crossref","first-page":"1935","DOI":"10.4049\/jimmunol.167.4.1935","article-title":"Somatic hypermutation and selection of B cells in thymic germinal centers responding to acetylcholine receptor in myasthenia gravis","volume":"167","author":"Sims","year":"2001","journal-title":"J. Immunol."},{"key":"ref_254","doi-asserted-by":"crossref","first-page":"1271","DOI":"10.1056\/NEJM197412122912403","article-title":"The thymus in myasthenia gravis. Evidence for altered cell populations","volume":"291","author":"Abdou","year":"1974","journal-title":"N. Engl. J. Med."},{"key":"ref_255","doi-asserted-by":"crossref","first-page":"2115","DOI":"10.4049\/jimmunol.145.7.2115","article-title":"Thymic B cells from myasthenia gravis patients are activated B cells. Phenotypic and functional analysis","volume":"145","author":"Leprince","year":"1990","journal-title":"J. Immunol."},{"key":"ref_256","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1097\/01.LAB.0000032379.63784.9C","article-title":"Antibodies to acetylcholine receptor in parous women with myasthenia: Evidence for immunization by fetal antigen","volume":"82","author":"Matthews","year":"2002","journal-title":"Lab. Investig."},{"key":"ref_257","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.jaut.2013.12.008","article-title":"Clonal heterogeneity of thymic B cells from early-onset myasthenia gravis patients with antibodies against the acetylcholine receptor","volume":"52","author":"Vrolix","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_258","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1196\/annals.1254.026","article-title":"Scenarios for autoimmunization of T and B cells in myasthenia gravis","volume":"998","author":"Shiono","year":"2003","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_259","doi-asserted-by":"crossref","first-page":"887","DOI":"10.4049\/jimmunol.136.3.887","article-title":"Specific activation of lymphocytes against acetylcholine receptor in the thymus in myasthenia gravis","volume":"136","author":"Fujii","year":"1986","journal-title":"J. Immunol."},{"key":"ref_260","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.jneuroim.2008.06.027","article-title":"The myasthenia gravis thymus: A rare source of human autoantibody-secreting plasma cells for testing potential therapeutics","volume":"201\u2013202","author":"Hill","year":"2008","journal-title":"J. Neuroimmunol."},{"key":"ref_261","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1016\/S0140-6736(78)90073-9","article-title":"In-vitro synthesis of anti-acetylcholine-receptor antibody by thymic lymphocytes in myasthenia gravis","volume":"1","author":"Vincent","year":"1978","journal-title":"Lancet"},{"key":"ref_262","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1212\/WNL.31.8.935","article-title":"Acetylcholine receptor antibody synthesis by thymic lymphocytes: Correlation with thymic histology","volume":"31","author":"Scadding","year":"1981","journal-title":"Neurology"},{"key":"ref_263","first-page":"378","article-title":"Greatly increased autoantibody production in myasthenia gravis by thymocyte suspensions prepared with proteolytic enzymes","volume":"54","author":"Willcox","year":"1983","journal-title":"Clin. Exp. Immunol."},{"key":"ref_264","first-page":"97","article-title":"Cell types required for anti-acetylcholine receptor antibody synthesis by cultured thymocytes and blood lymphocytes in myasthenia gravis","volume":"58","author":"Willcox","year":"1984","journal-title":"Clin. Exp. Immunol."},{"key":"ref_265","first-page":"979","article-title":"Isolation of germinal centre (GC) cells is greatly improved by using the protease dispase to prepare cell suspensions","volume":"186","author":"Willcox","year":"1985","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_266","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/0165-5728(89)90119-7","article-title":"In vitro synthesis of antibodies to acetylcholine receptor by Epstein-Barr virus-stimulated B-lymphocytes derived from patients with myasthenia gravis","volume":"24","author":"Brenner","year":"1989","journal-title":"J. Neuroimmunol."},{"key":"ref_267","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1038\/310244a0","article-title":"Autoimmune human T lymphocytes specific for acetylcholine receptor","volume":"310","author":"Hohlfeld","year":"1984","journal-title":"Nature"},{"key":"ref_268","doi-asserted-by":"crossref","first-page":"2268","DOI":"10.1172\/JCI2068","article-title":"A pathogenetic role for the thymoma in myasthenia gravis. Autosensitization of IL-4- producing T cell clones recognizing extracellular acetylcholine receptor epitopes presented by minority class II isotypes","volume":"101","author":"Nagvekar","year":"1998","journal-title":"J. Clin. Investig."},{"key":"ref_269","doi-asserted-by":"crossref","first-page":"3872","DOI":"10.1182\/blood.V96.12.3872","article-title":"Thymomas alter the T-cell subset composition in the blood: A potential mechanism for thymoma-associated autoimmune disease","volume":"96","author":"Hoffacker","year":"2000","journal-title":"Blood"},{"key":"ref_270","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1002\/ana.1017","article-title":"Mature, long-lived CD4+ and CD8+ T cells are generated by the thymoma in myasthenia gravis","volume":"50","author":"Buckley","year":"2001","journal-title":"Ann. Neurol."},{"key":"ref_271","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1182\/blood.V100.1.159","article-title":"Paraneoplastic myasthenia gravis correlates with generation of mature naive CD4(+) T cells in thymomas","volume":"100","author":"Strobel","year":"2002","journal-title":"Blood"},{"key":"ref_272","doi-asserted-by":"crossref","first-page":"314","DOI":"10.21037\/jtd.2016.03.03","article-title":"Increased frequency of thymic T follicular helper cells in myasthenia gravis patients with thymoma","volume":"8","author":"Song","year":"2016","journal-title":"J. Thorac. Dis."},{"key":"ref_273","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1007\/s12035-014-8985-1","article-title":"Intrathymic Tfh\/B Cells Interaction Leads to Ectopic GCs Formation and Anti-AChR Antibody Production: Central Role in Triggering MG Occurrence","volume":"53","author":"Zhang","year":"2016","journal-title":"Mol. Neurobiol."},{"key":"ref_274","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1038\/nm1551","article-title":"A brief history of T(H)17, the first major revision in the T(H)1\/T(H)2 hypothesis of T cell-mediated tissue damage","volume":"13","author":"Steinman","year":"2007","journal-title":"Nat. Med."},{"key":"ref_275","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1002\/mus.22070","article-title":"Increased serum interleukin-17 levels in patients with myasthenia gravis","volume":"44","author":"Roche","year":"2011","journal-title":"Muscle Nerve"},{"key":"ref_276","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1111\/j.1365-3083.2012.02703.x","article-title":"T helper type 17 cells expand in patients with myasthenia-associated thymoma","volume":"76","author":"Wang","year":"2012","journal-title":"Scand. J. Immunol."},{"key":"ref_277","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.jaut.2013.12.015","article-title":"Both Treg cells and Tconv cells are defective in the Myasthenia gravis thymus: Roles of IL-17 and TNF-alpha","volume":"52","author":"Gradolatto","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_278","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1016\/j.jaut.2013.12.005","article-title":"Characterization of CD4 and CD8 T cell responses in MuSK myasthenia gravis","volume":"52","author":"Yi","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_279","doi-asserted-by":"crossref","unstructured":"Schaffert, H., Pelz, A., Saxena, A., Losen, M., Meisel, A., Thiel, A., and Kohler, S. (2015). IL-17-producing CD4 T cells contribute to the loss of B-cell tolerance in experimental autoimmune myasthenia gravis. Eur. J. Immunol.","DOI":"10.1002\/eji.201445064"},{"key":"ref_280","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.jaut.2018.11.005","article-title":"Il-23\/Th17 cell pathway: A promising target to alleviate thymic inflammation maintenance in myasthenia gravis","volume":"98","author":"Villegas","year":"2019","journal-title":"J. Autoimmun."},{"key":"ref_281","doi-asserted-by":"crossref","first-page":"2075","DOI":"10.4049\/jimmunol.1501339","article-title":"Autoreactive T Cells from Patients with Myasthenia Gravis Are Characterized by Elevated IL-17, IFN-gamma, and GM-CSF and Diminished IL-10 Production","volume":"196","author":"Cao","year":"2016","journal-title":"J. Immunol."},{"key":"ref_282","doi-asserted-by":"crossref","first-page":"201","DOI":"10.3109\/08916934.2014.992517","article-title":"B cells produce less IL-10, IL-6 and TNF-alpha in myasthenia gravis","volume":"48","author":"Yilmaz","year":"2015","journal-title":"Autoimmunity"},{"key":"ref_283","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1111\/nyas.13591","article-title":"An imbalance between regulatory T cells and T helper 17 cells in acetylcholine receptor-positive myasthenia gravis patients","volume":"1413","author":"Villegas","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_284","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.jaut.2013.06.006","article-title":"Treg and CTLA-4: Two intertwining pathways to immune tolerance","volume":"45","author":"Walker","year":"2013","journal-title":"J. Autoimmun."},{"key":"ref_285","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.jaut.2012.12.003","article-title":"T-regs in autoimmune hepatitis-systemic lupus erythematosus\/mixed connective tissue disease overlap syndrome are functionally defective and display a Th1 cytokine profile","volume":"41","author":"Longhi","year":"2013","journal-title":"J. Autoimmun."},{"key":"ref_286","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1111\/imr.12169","article-title":"Regulatory T cells in autoimmune neuroinflammation","volume":"259","author":"Kleinewietfeld","year":"2014","journal-title":"Immunol. Rev."},{"key":"ref_287","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1182\/blood-2003-11-3900","article-title":"Functional defect of regulatory CD4(+)CD25+ T cells in the thymus of patients with autoimmune myasthenia gravis","volume":"105","author":"Balandina","year":"2005","journal-title":"Blood"},{"key":"ref_288","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1016\/j.clim.2012.09.012","article-title":"Impaired regulatory function in circulating CD4(+)CD25(high)CD127(low\/-) T cells in patients with myasthenia gravis","volume":"145","author":"Thiruppathi","year":"2012","journal-title":"Clin. Immunol."},{"key":"ref_289","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1111\/j.1365-2567.2005.02220.x","article-title":"Circulating and thymic CD4 CD25 T regulatory cells in myasthenia gravis: Effect of immunosuppressive treatment","volume":"116","author":"Fattorossi","year":"2005","journal-title":"Immunology"},{"key":"ref_290","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.imlet.2008.11.015","article-title":"The role of FoxP3+CD4+CD25hi Tregs in the pathogenesis of myasthenia gravis","volume":"122","author":"Zhang","year":"2009","journal-title":"Immunol. Lett."},{"key":"ref_291","doi-asserted-by":"crossref","unstructured":"Nishimura, T., Inaba, Y., Nakazawa, Y., Omata, T., Akasaka, M., Shirai, I., and Ichikawa, M. (2015). Reduction in peripheral regulatory T cell population in childhood ocular type myasthenia gravis. Brain Dev.","DOI":"10.1016\/j.braindev.2014.12.007"},{"key":"ref_292","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/j.jneuroim.2005.03.011","article-title":"Decreased frequency of intrathymic regulatory T cells in patients with myasthenia-associated thymoma","volume":"164","author":"Luther","year":"2005","journal-title":"J. Neuroimmunol."},{"key":"ref_293","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1007\/s10875-007-9147-2","article-title":"Thymopoiesis, regulatory T cells, and TCRVbeta expression in thymoma with and without myasthenia gravis, and modulatory effects of steroid therapy","volume":"28","author":"Fattorossi","year":"2008","journal-title":"J. Clin. Immunol."},{"key":"ref_294","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1196\/annals.1405.021","article-title":"Autoimmunizing mechanisms in thymoma and thymus","volume":"1132","author":"Willcox","year":"2008","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_295","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1111\/nyas.13560","article-title":"Pathophysiological mechanisms of autoimmunity","volume":"1413","author":"Sudres","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_296","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1111\/nyas.13510","article-title":"Circulating microRNAs as potential biomarkers in myasthenia gravis patients","volume":"1412","author":"Punga","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_297","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1002\/mus.25973","article-title":"B cells in the pathophysiology of myasthenia gravis","volume":"57","author":"Yi","year":"2018","journal-title":"Muscle Nerve"},{"key":"ref_298","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1016\/j.ncl.2018.01.011","article-title":"Treatment of Myasthenia Gravis","volume":"36","author":"Farmakidis","year":"2018","journal-title":"Neurol. Clin."},{"key":"ref_299","doi-asserted-by":"crossref","first-page":"265","DOI":"10.3233\/JND-170294","article-title":"New Pathways and Therapeutic Targets in Autoimmune Myasthenia Gravis","volume":"5","author":"Behin","year":"2018","journal-title":"J. Neuromuscul. Dis."},{"key":"ref_300","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1038\/s41582-018-0110-z","article-title":"Immunotherapy in myasthenia gravis in the era of biologics","volume":"15","author":"Dalakas","year":"2019","journal-title":"Nat. Rev. Neurol."},{"key":"ref_301","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.thorsurg.2018.12.002","article-title":"A Neurologist\u2019s Perspective on Understanding Myasthenia Gravis: Clinical Perspectives of Etiologic Factors, Diagnosis, and Preoperative Treatment","volume":"29","author":"Mukharesh","year":"2019","journal-title":"Thorac. Surg. Clin."},{"key":"ref_302","doi-asserted-by":"crossref","first-page":"1373","DOI":"10.1016\/S0140-6736(76)91917-6","article-title":"Remission of myasthenia gravis following plasma-exchange","volume":"2","author":"Pinching","year":"1976","journal-title":"Lancet"},{"key":"ref_303","first-page":"848","article-title":"High-dose intravenous gammaglobulin for myasthenia gravis","volume":"1","author":"Wick","year":"1984","journal-title":"Lancet"},{"key":"ref_304","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1016\/S1474-4422(17)30369-1","article-title":"Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): A phase 3, randomised, double-blind, placebo-controlled, multicentre study","volume":"16","author":"Howard","year":"2017","journal-title":"Lancet Neurol."},{"key":"ref_305","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1002\/mus.26447","article-title":"Long-term safety and efficacy of eculizumab in generalized myasthenia gravis","volume":"60","author":"Muppidi","year":"2019","journal-title":"Muscle Nerve"},{"key":"ref_306","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1002\/mus.23839","article-title":"A randomized, double-blind, placebo-controlled phase II study of eculizumab in patients with refractory generalized myasthenia gravis","volume":"48","author":"Howard","year":"2013","journal-title":"Muscle Nerve"},{"key":"ref_307","doi-asserted-by":"crossref","first-page":"1756285617749134","DOI":"10.1177\/1756285617749134","article-title":"When myasthenia gravis is deemed refractory: Clinical signposts and treatment strategies","volume":"11","author":"Mantegazza","year":"2018","journal-title":"Ther. Adv. Neurol. Disord."},{"key":"ref_308","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1111\/nyas.13555","article-title":"Acetylcholine receptor antibody-mediated animal models of myasthenia gravis and the role of complement","volume":"1413","author":"Kusner","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_309","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1007\/s40265-019-01136-7","article-title":"Risankizumab: First Global Approval","volume":"79","author":"McKeage","year":"2019","journal-title":"Drugs"},{"key":"ref_310","doi-asserted-by":"crossref","unstructured":"Zelek, W.M., Taylor, P.R., and Morgan, B.P. (2019). Development and characterization of novel anti-C5 monoclonal antibodies capable of inhibiting complement in multiple species. Immunology.","DOI":"10.3389\/fimmu.2020.612402"},{"key":"ref_311","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.4049\/jimmunol.1301555","article-title":"Proteasome inhibition with bortezomib depletes plasma cells and specific autoantibody production in primary thymic cell cultures from early-onset myasthenia gravis patients","volume":"193","author":"Gomez","year":"2014","journal-title":"J. Immunol."},{"key":"ref_312","doi-asserted-by":"crossref","first-page":"2503","DOI":"10.4049\/jimmunol.1002539","article-title":"Proteasome inhibition with bortezomib depletes plasma cells and autoantibodies in experimental autoimmune myasthenia gravis","volume":"186","author":"Gomez","year":"2011","journal-title":"J. Immunol."},{"key":"ref_313","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1002\/art.21617","article-title":"Reconstitution of peripheral blood B cells after depletion with rituximab in patients with rheumatoid arthritis","volume":"54","author":"Leandro","year":"2006","journal-title":"Arthritis Rheum."},{"key":"ref_314","doi-asserted-by":"crossref","first-page":"4658","DOI":"10.1073\/pnas.1001074107","article-title":"Rituximab specifically depletes short-lived autoreactive plasma cells in a mouse model of inflammatory arthritis","volume":"107","author":"Huang","year":"2010","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_315","doi-asserted-by":"crossref","first-page":"5105","DOI":"10.4049\/jimmunol.1202317","article-title":"Pathogenic Long-Lived Plasma Cells and Their Survival Niches in Autoimmunity, Malignancy, and Allergy","volume":"189","author":"Winter","year":"2012","journal-title":"J. Immunol."},{"key":"ref_316","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1002\/mus.20479","article-title":"Successful treatment of MuSK antibody-positive myasthenia gravis with rituximab","volume":"33","author":"Hain","year":"2006","journal-title":"Muscle Nerve"},{"key":"ref_317","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1016\/j.nmd.2007.06.196","article-title":"Successful long-term treatment of MuSK antibody-positive myasthenia gravis with rituximab, a follow up","volume":"17","author":"Jordan","year":"2007","journal-title":"Neuromuscul. Disord."},{"key":"ref_318","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1212\/WNL.0b013e3182407982","article-title":"Long-lasting treatment effect of rituximab in MuSK myasthenia","volume":"78","author":"Querol","year":"2012","journal-title":"Neurology"},{"key":"ref_319","doi-asserted-by":"crossref","first-page":"992","DOI":"10.1002\/mus.24063","article-title":"Prolonged B-Cell Depletion in Musk Myasthenia Gravis Following Rituximab Treatment","volume":"48","author":"Yi","year":"2013","journal-title":"Muscle Nerve"},{"key":"ref_320","first-page":"519","article-title":"Sustained remission in a case of musk (+) myasthenia gravis treated with i.v. rituximab as primary therapy","volume":"21","author":"Cupler","year":"2014","journal-title":"Eur. J. Neurol."},{"key":"ref_321","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1067\/S0022-3476(03)00300-7","article-title":"Successful treatment of refractory myasthenia gravis using rituximab: A pediatric case report","volume":"143","author":"Wylam","year":"2003","journal-title":"J. Pediatr."},{"key":"ref_322","doi-asserted-by":"crossref","first-page":"1069","DOI":"10.1212\/WNL.0000000000004341","article-title":"Rituximab as treatment for anti-MuSK myasthenia gravis: Multicenter blinded prospective review","volume":"89","author":"Hehir","year":"2017","journal-title":"Neurology"},{"key":"ref_323","doi-asserted-by":"crossref","first-page":"1115","DOI":"10.1007\/s00415-014-7532-3","article-title":"Efficacy and safety of rituximab for myasthenia gravis: A systematic review and meta-analysis","volume":"262","author":"Iorio","year":"2015","journal-title":"J. Neurol."},{"key":"ref_324","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1016\/j.pediatrneurol.2012.05.017","article-title":"Rituximab in severe seronegative juvenile myasthenia gravis: Review of the literature","volume":"47","author":"Koul","year":"2012","journal-title":"Pediatr. Neurol."},{"key":"ref_325","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1136\/jnnp.2010.220475","article-title":"Use and monitoring of low dose rituximab in myasthenia gravis","volume":"82","author":"Blum","year":"2011","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_326","doi-asserted-by":"crossref","first-page":"968","DOI":"10.1007\/s00415-006-0442-2","article-title":"Rituximab in refractory MuSK antibody myasthenia gravis","volume":"254","author":"Thakre","year":"2007","journal-title":"J. Neurol."},{"key":"ref_327","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1136\/jnnp.2006.093567","article-title":"Complete remission induced by rituximab in refractory, seronegative, muscle-specific, kinase-positive myasthenia gravis","volume":"78","author":"Baek","year":"2007","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_328","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.jneuroim.2008.04.039","article-title":"Sustained response to Rituximab in anti-AChR and anti-MuSK positive Myasthenia Gravis patients","volume":"201\u2013202","author":"Illa","year":"2008","journal-title":"J. Neuroimmunol."},{"key":"ref_329","doi-asserted-by":"crossref","unstructured":"Keung, B., Robeson, K., DiCapua, D.B., Rosen, J.B., O\u2019Connor, K.C., Goldstein, J.M., and Nowak, R.J. (2013). Long term Efficacy of Rituximab in Patients with MuSK Autoantibody Myasthenia Gravis. J. Neurol. Neurosurg. Psychiatry.","DOI":"10.1136\/jnnp-2012-303664"},{"key":"ref_330","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1007\/s40265-019-1065-0","article-title":"Therapies Directed Against B-Cells and Downstream Effectors in Generalized Autoimmune Myasthenia Gravis: Current Status","volume":"79","author":"Beecher","year":"2019","journal-title":"Drugs"},{"key":"ref_331","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1111\/nyas.13550","article-title":"Acetylcholine receptor-specific immunosuppressive therapy of experimental autoimmune myasthenia gravis and myasthenia gravis","volume":"1413","author":"Luo","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_332","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1196\/annals.1405.017","article-title":"Antigen-specific apheresis of pathogenic autoantibodies from myasthenia gravis sera","volume":"1132","author":"Tzartos","year":"2008","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_333","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1002\/eji.201444993","article-title":"Selection and depletion of plasma cells based on the specificity of the secreted antibody","volume":"45","author":"Taddeo","year":"2015","journal-title":"Eur. J. Immunol."},{"key":"ref_334","doi-asserted-by":"crossref","first-page":"992","DOI":"10.1038\/s41598-017-01019-5","article-title":"Hinge-deleted IgG4 blocker therapy for acetylcholine receptor myasthenia gravis in rhesus monkeys","volume":"7","author":"Losen","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_335","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1212\/WNL.0000000000002790","article-title":"International consensus guidance for management of myasthenia gravis: Executive summary","volume":"87","author":"Sanders","year":"2016","journal-title":"Neurology"},{"key":"ref_336","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1196\/annals.1405.012","article-title":"Response to therapy in myasthenia gravis with anti-MuSK antibodies","volume":"1132","author":"Evoli","year":"2008","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_337","doi-asserted-by":"crossref","first-page":"1508","DOI":"10.1212\/01.wnl.0000183145.91579.74","article-title":"Nonresponsiveness to anticholinesterase agents in patients with MuSK-antibody-positive MG","volume":"65","author":"Hatanaka","year":"2005","journal-title":"Neurology"},{"key":"ref_338","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1016\/j.neuron.2005.04.002","article-title":"Neurotransmitter acetylcholine negatively regulates neuromuscular synapse formation by a Cdk5-dependent mechanism","volume":"46","author":"Lin","year":"2005","journal-title":"Neuron"},{"key":"ref_339","doi-asserted-by":"crossref","first-page":"11088","DOI":"10.1073\/pnas.0504806102","article-title":"Agrin promotes synaptic differentiation by counteracting an inhibitory effect of neurotransmitter","volume":"102","author":"Misgeld","year":"2005","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_340","doi-asserted-by":"crossref","first-page":"119","DOI":"10.3988\/jcn.2014.10.2.119","article-title":"Clinical and Electrophysiologic Responses to Acetylcholinesterase Inhibitors in MuSK-Antibody-Positive Myasthenia Gravis: Evidence for Cholinergic Neuromuscular Hyperactivity","volume":"10","author":"Shin","year":"2014","journal-title":"J. Clin. Neurol."},{"key":"ref_341","doi-asserted-by":"crossref","first-page":"1819","DOI":"10.1212\/WNL.0b013e318237f660","article-title":"Anti-MuSK autoantibodies block binding of collagen Q to MuSK","volume":"77","author":"Kawakami","year":"2011","journal-title":"Neurology"},{"key":"ref_342","doi-asserted-by":"crossref","first-page":"S36","DOI":"10.1016\/S0960-8966(17)30325-5","article-title":"Inhibition of the tyrosine phosphatase Shp2 alleviates the pathogeniceff ects of MuSK antibodies in vitro","volume":"27","author":"Huda","year":"2017","journal-title":"Neuromuscul. Disord."},{"key":"ref_343","doi-asserted-by":"crossref","first-page":"2559","DOI":"10.1016\/j.ajpath.2016.05.025","article-title":"Silencing of Dok-7 in Adult Rat Muscle Increases Susceptibility to Passive Transfer Myasthenia Gravis","volume":"186","author":"Gomez","year":"2016","journal-title":"Am. J. Pathol."},{"key":"ref_344","doi-asserted-by":"crossref","first-page":"1800","DOI":"10.1101\/gad.375206","article-title":"Casein kinase 2-dependent serine phosphorylation of MuSK regulates acetylcholine receptor aggregation at the neuromuscular junction","volume":"20","author":"Cheusova","year":"2006","journal-title":"Genes Dev."},{"key":"ref_345","doi-asserted-by":"crossref","first-page":"2327","DOI":"10.1093\/brain\/awh612","article-title":"Increased expression of rapsyn in muscles prevents acetylcholine receptor loss in experimental autoimmune myasthenia gravis","volume":"128","author":"Losen","year":"2005","journal-title":"Brain"},{"key":"ref_346","doi-asserted-by":"crossref","first-page":"644","DOI":"10.2353\/ajpath.2007.060676","article-title":"Overexpression of rapsyn in rat muscle increases acetylcholine receptor levels in chronic experimental autoimmune myasthenia gravis","volume":"170","author":"Losen","year":"2007","journal-title":"Am. J. Pathol."},{"key":"ref_347","doi-asserted-by":"crossref","first-page":"814","DOI":"10.1002\/mus.26025","article-title":"Engineered agrin attenuates the severity of experimental autoimmune myasthenia gravis","volume":"57","author":"Li","year":"2018","journal-title":"Muscle Nerve"},{"key":"ref_348","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1016\/j.scr.2015.07.005","article-title":"Formation and characterisation of neuromuscular junctions between hiPSC derived motoneurons and myotubes","volume":"15","author":"Demestre","year":"2015","journal-title":"Stem Cell Res."},{"key":"ref_349","doi-asserted-by":"crossref","first-page":"9602","DOI":"10.1016\/j.biomaterials.2011.09.014","article-title":"Neuromuscular junction formation between human stem cell-derived motoneurons and human skeletal muscle in a defined system","volume":"32","author":"Guo","year":"2011","journal-title":"Biomaterials"},{"key":"ref_350","doi-asserted-by":"crossref","first-page":"9413","DOI":"10.1016\/j.biomaterials.2013.08.062","article-title":"Three-dimensional neuron-muscle constructs with neuromuscular junctions","volume":"34","author":"Morimoto","year":"2013","journal-title":"Biomaterials"},{"key":"ref_351","doi-asserted-by":"crossref","first-page":"eaat5847","DOI":"10.1126\/sciadv.aat5847","article-title":"Microphysiological 3D model of amyotrophic lateral sclerosis (ALS) from human iPS-derived muscle cells and optogenetic motor neurons","volume":"4","author":"Osaki","year":"2018","journal-title":"Sci. Adv."},{"key":"ref_352","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1001\/jamaneurol.2015.0203","article-title":"Clinical Features and Diagnostic Usefulness of Antibodies to Clustered Acetylcholine Receptors in the Diagnosis of Seronegative Myasthenia Gravis","volume":"72","author":"Huda","year":"2015","journal-title":"JAMA Neurol."},{"key":"ref_353","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1111\/ene.12270","article-title":"Antibodies to clustered acetylcholine receptor: Expanding the phenotype","volume":"21","author":"Devic","year":"2014","journal-title":"Eur. J. Neurol."},{"key":"ref_354","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1093\/brain\/awn092","article-title":"IgG1 antibodies to acetylcholine receptors in \u2018seronegative\u2019 myasthenia gravis","volume":"131","author":"Leite","year":"2008","journal-title":"Brain"},{"key":"ref_355","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1111\/cen3.12107","article-title":"Evaluation of aquaporin-4 antibody assays","volume":"5","author":"Waters","year":"2014","journal-title":"Clin. Exp. Neuroimmunol."},{"key":"ref_356","doi-asserted-by":"crossref","first-page":"e357","DOI":"10.1212\/NXI.0000000000000357","article-title":"IgG-specific cell-based assay detects potentially pathogenic MuSK-Abs in seronegative MG","volume":"4","author":"Huda","year":"2017","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_357","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1111\/j.1749-6632.2012.06833.x","article-title":"The search for new antigenic targets in myasthenia gravis","volume":"1275","author":"Cossins","year":"2012","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_358","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1111\/j.1749-6632.2012.06789.x","article-title":"Antibodies identified by cell-based assays in myasthenia gravis and associated diseases","volume":"1274","author":"Vincent","year":"2012","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_359","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1111\/nyas.13573","article-title":"Agrin and LRP4 antibodies as new biomarkers of myasthenia gravis","volume":"1413","author":"Yan","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_360","doi-asserted-by":"crossref","first-page":"3749","DOI":"10.1016\/j.febslet.2013.10.001","article-title":"Mesdc2 plays a key role in cell-surface expression of Lrp4 and postsynaptic specialization in myotubes","volume":"587","author":"Hoshi","year":"2013","journal-title":"FEBS Lett."},{"key":"ref_361","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1002\/mus.25438","article-title":"Agrin and low-density lipoprotein-related receptor protein 4 antibodies in amyotrophic lateral sclerosis patients","volume":"55","author":"Rivner","year":"2017","journal-title":"Muscle Nerve"},{"key":"ref_362","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1002\/acn3.26","article-title":"LRP4 antibodies in serum and CSF from amyotrophic lateral sclerosis patients","volume":"1","author":"Tzartos","year":"2014","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_363","doi-asserted-by":"crossref","first-page":"3021","DOI":"10.2169\/internalmedicine.0966-18","article-title":"Late-onset Myasthenia Gravis Accompanied by Amyotrophic Lateral Sclerosis with Antibodies against the Acetylcholine Receptor and Low-density Lipoprotein Receptor-related Protein 4","volume":"57","author":"Ohnari","year":"2018","journal-title":"Intern. Med."},{"key":"ref_364","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1097\/CND.0b013e31824db163","article-title":"Amyotrophic lateral sclerosis with positive anti-acetylcholine receptor antibodies. Case report and review of the literature","volume":"14","author":"Mehanna","year":"2012","journal-title":"J. Clin. Neuromuscul. Dis."},{"key":"ref_365","doi-asserted-by":"crossref","first-page":"312","DOI":"10.2169\/internalmedicine.36.312","article-title":"Amyotrophic lateral sclerosis with anti-acetylcholine receptor antibody","volume":"36","author":"Okuyama","year":"1997","journal-title":"Intern. Med."}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/8\/7\/671\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:01:57Z","timestamp":1760187717000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/8\/7\/671"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,7,2]]},"references-count":365,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2019,7]]}},"alternative-id":["cells8070671"],"URL":"https:\/\/doi.org\/10.3390\/cells8070671","relation":{},"ISSN":["2073-4409"],"issn-type":[{"value":"2073-4409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,7,2]]}}}