{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T06:26:20Z","timestamp":1790663180208,"version":"4.1.0"},"reference-count":218,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2020,10,26]],"date-time":"2020-10-26T00:00:00Z","timestamp":1603670400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pharmaceuticals"],"abstract":"<jats:p>Precision medicine has emerged as a central element of healthcare science. Complement, a component of innate immunity known for centuries, has been implicated in the pathophysiology of numerous incurable neurological diseases, emerging as a potential therapeutic target and predictive biomarker. In parallel, the innovative application of the first complement inhibitor in clinical practice as an approved treatment of myasthenia gravis (MG) and neuromyelitis optica spectrum disorders (NMOSD) related with specific antibodies raised hope for the implementation of personalized therapies in detrimental neurological diseases. A thorough literature search was conducted through May 2020 at MEDLINE, EMBASE, Cochrane Library and ClinicalTrials.gov databases based on medical terms (MeSH)\u201d complement system proteins\u201d and \u201cneurologic disease\u201d. Complement\u2019s role in pathophysiology, monitoring of disease activity and therapy has been investigated in MG, multiple sclerosis, NMOSD, spinal muscular atrophy, amyotrophic lateral sclerosis, Parkinson, Alzheimer, Huntington disease, Guillain\u2013Barr\u00e9 syndrome, chronic inflammatory demyelinating polyneuropathy, stroke, and epilepsy. Given the complexity of complement diagnostics and therapeutics, this state-of-the-art review aims to provide a brief description of the complement system for the neurologist, an overview of novel complement inhibitors and updates of complement studies in a wide range of neurological disorders.<\/jats:p>","DOI":"10.3390\/ph13110341","type":"journal-article","created":{"date-parts":[[2020,10,26]],"date-time":"2020-10-26T10:38:35Z","timestamp":1603708715000},"page":"341","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Precision Medicine in Neurology: The Inspirational Paradigm of Complement Therapeutics"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1958-0063","authenticated-orcid":false,"given":"Maria","family":"Gavriilaki","sequence":"first","affiliation":[{"name":"Postgraduate Course, School of Medicine, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vasilios K.","family":"Kimiskidis","sequence":"additional","affiliation":[{"name":"Postgraduate Course, School of Medicine, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece"},{"name":"Laboratory of Clinical Neurophysiology, AHEPA Hospital, Aristotle University of Thessaloniki, 54621 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8883-8208","authenticated-orcid":false,"given":"Eleni","family":"Gavriilaki","sequence":"additional","affiliation":[{"name":"Hematology Department-BMT Unit, G. Papanicolaou Hospital, 57010 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,10,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1111\/j.1749-6632.1999.tb08532.x","article-title":"The human genome project and the future of medicine","volume":"882","author":"Collins","year":"1999","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"946","DOI":"10.1172\/JCI126119","article-title":"Promises, promises, and precision medicine","volume":"129","author":"Joyner","year":"2019","journal-title":"J. Clin. Investig."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Zelek, W.M., Fathalla, D., Morgan, A., Touchard, S., Loveless, S., Tallantyre, E., Robertson, N.P., and Morgan, B.P. (2019). Cerebrospinal fluid complement system biomarkers in demyelinating disease. Mult. Scler. J., 2019.","DOI":"10.1177\/1352458519887905"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.eplepsyres.2017.02.001","article-title":"Real-world utility of whole exome sequencing with targeted gene analysis for focal epilepsy","volume":"131","author":"Perucca","year":"2017","journal-title":"Epilepsy Res."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Burgos, K., Malenica, I., Metpally, R., Courtright, A., Rakela, B., Beach, T., Shill, H., Adler, C., Sabbagh, M., and Villa, S. (2014). Profiles of extracellular miRNA in cerebrospinal fluid and serum from patients with Alzheimer\u2019s and Parkinson\u2019s diseases correlate with disease status and features of pathology. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0094839"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/bs.ctdb.2018.03.002","article-title":"Studying the Brain in a Dish: 3D Cell Culture Models of Human Brain Development and Disease","volume":"129","author":"Brown","year":"2018","journal-title":"Curr. Top. Dev. Biol."},{"key":"ref_7","unstructured":"Szab\u00f3, C.D., Tak\u00e1cs, B., and Proh\u00e1szka, Z. (September, January 30). Complement terminal pathway deficiency in a patient with recurrent meningitis. Proceedings of the 59th National Congress of the Hungarian Society of Laboratory Medicine P\u00e9cs, Hungary: Clinical Chemistry and Laboratory Medicine, Montr\u00e9al, QC, Canada."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1056\/NEJMoa061648","article-title":"The Complement Inhibitor Eculizumab in Paroxysmal Nocturnal Hemoglobinuria","volume":"355","author":"Hillmen","year":"2006","journal-title":"N. Engl. J. Med."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1840","DOI":"10.1182\/blood-2007-06-094136","article-title":"Multicenter phase 3 study of the complement inhibitor eculizumab for the treatment of patients with paroxysmal nocturnal hemoglobinuria","volume":"111","author":"Brodsky","year":"2008","journal-title":"Blood"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"337","DOI":"10.3389\/fimmu.2019.00337","article-title":"Complement in Thrombotic Microangiopathies: Unraveling Ariadne\u2019s Thread Into the Labyrinth of Complement Therapeutics","volume":"10","author":"Gavriilaki","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.blre.2017.02.003","article-title":"Complementopathies","volume":"31","author":"Baines","year":"2017","journal-title":"Blood Rev."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1053\/j.ajkd.2015.11.009","article-title":"Discontinuation of Eculizumab in a Patient with Atypical Hemolytic Uremic Syndrome Due to a Mutation in CFH","volume":"67","author":"Habbig","year":"2016","journal-title":"Am. J. Kidney Dis."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"632","DOI":"10.1056\/NEJMoa1311084","article-title":"Genetic Variants in C5 and Poor Response to Eculizumab","volume":"370","author":"Nishimura","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1038\/nri3547","article-title":"Age-dependent dysregulation of innate immunity","volume":"13","author":"Shaw","year":"2013","journal-title":"Nat. Rev. Immunol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.1016\/j.cell.2007.10.036","article-title":"The Classical Complement Cascade Mediates CNS Synapse Elimination","volume":"131","author":"Stevens","year":"2007","journal-title":"Cell"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1016\/j.neuron.2012.03.026","article-title":"Microglia Sculpt Postnatal Neural Circuits in an Activity and Complement-Dependent Manner","volume":"74","author":"Schafer","year":"2012","journal-title":"Neuron"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1038\/nature21029","article-title":"Neurotoxic reactive astrocytes are induced by activated microglia","volume":"541","author":"Liddelow","year":"2017","journal-title":"Nature"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"25","DOI":"10.2174\/187152711794488601","article-title":"Activation and Control of CNS Innate Immune Responses in Health and Diseases: A Balancing Act Finely Tuned by Neuroimmune Regulators (NIReg)","volume":"10","author":"Hoarau","year":"2011","journal-title":"CNS Neurol. Disord. Drug Targets"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1146\/annurev-neuro-061010-113810","article-title":"The Complement System: An Unexpected Role in Synaptic Pruning During Development and Disease","volume":"35","author":"Stephan","year":"2012","journal-title":"Annu. Rev. Neurosci."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1016\/j.immuni.2019.03.016","article-title":"Immune Signaling in Neurodegeneration","volume":"50","author":"Hammond","year":"2019","journal-title":"Immunity"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"13460","DOI":"10.1523\/JNEUROSCI.1333-13.2013","article-title":"A Dramatic Increase of C1q Protein in the CNS during Normal Aging","volume":"33","author":"Stephan","year":"2013","journal-title":"J. Neurosci."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"712","DOI":"10.1126\/science.aad8373","article-title":"Complement and microglia mediate early synapse loss in Alzheimer mouse models","volume":"352","author":"Hong","year":"2016","journal-title":"Science"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/j.molimm.2018.06.267","article-title":"Blood-brain barrier (BBB) and the complement landscape","volume":"102","author":"Alexander","year":"2018","journal-title":"Mol. Immunol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1155\/2018\/2530414","article-title":"Contribution of Neurons and Glial Cells to Complement-Mediated Synapse Removal during Development, Aging and in Alzheimer\u2019s Disease","volume":"2018","author":"Luchena","year":"2018","journal-title":"Mediat. Inflamm."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"362","DOI":"10.3389\/fimmu.2019.00362","article-title":"Therapeutic Inhibition of the Complement System in Diseases of the Central Nervous System","volume":"10","author":"Carpanini","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1016\/j.hoc.2015.02.001","article-title":"Complement: An Overview for the Clinician","volume":"29","author":"Varela","year":"2015","journal-title":"Hematol. Clin. N. Am."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1058","DOI":"10.1056\/NEJM200104053441406","article-title":"Complement. First of two parts","volume":"344","author":"Walport","year":"2001","journal-title":"N. Engl. J. Med."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1111\/j.1582-4934.2008.00350.x","article-title":"The alternative complement pathway revisited","volume":"12","author":"Harboe","year":"2008","journal-title":"J. Cell. Mol. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1016\/0161-5890(79)90089-0","article-title":"Isotypes of IgG: Comparison of the primary structures of three pairs of isotypes which differ in their ability to activate complement","volume":"16","author":"Brunhouse","year":"1979","journal-title":"Mol. Immunol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1016\/0090-1229(82)90116-7","article-title":"Detection and analysis of inborn and acquired complement abnormalities","volume":"23","author":"Gewurz","year":"1982","journal-title":"Clin. Immunol. Immunopathol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/S0162-3109(99)00007-7","article-title":"Regulation of complement activation by C-reactive protein","volume":"42","author":"Mold","year":"1999","journal-title":"Immunopharmacology"},{"key":"ref_32","first-page":"412","article-title":"Local release of properdin in the cellular microenvironment: Role in pattern recognition and amplification of the alternative pathway of complement","volume":"3","author":"Cortes","year":"2012","journal-title":"Front. Immunol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1111\/j.1749-6632.1983.tb18116.x","article-title":"Initiation of the alternative complement pathway due to spontaneous hydrolysis of the thioester of C3","volume":"421","author":"Pangburn","year":"1983","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1128\/IAI.68.2.688-693.2000","article-title":"Mannose-Binding Lectin Binds to a Range of Clinically Relevant Microorganisms and Promotes Complement Deposition","volume":"68","author":"Neth","year":"2000","journal-title":"Infect. Immun."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1099\/0022-1317-81-4-949","article-title":"Interaction of mannose-binding lectin with primary isolates of human immunodeficiency virus type 1","volume":"81","author":"Saifuddin","year":"2000","journal-title":"J. Gen. Virol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"10498","DOI":"10.1073\/pnas.1202588109","article-title":"Revised mechanism of complement lectin-pathway activation revealing the role of serine protease MASP-1 as the exclusive activator of MASP-2","volume":"109","author":"Kocsis","year":"2012","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"445","DOI":"10.4049\/jimmunol.135.1.445","article-title":"The membrane attack complex of complement: Relation of C7 to the metastable membrane binding site of the intermediate complex C5b-7","volume":"135","author":"Preissner","year":"1985","journal-title":"J. Immunol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1055\/s-0039-1692721","article-title":"Linking Complement Activation, Coagulation, and Neutrophils in Transplant-Associated Thrombotic Microangiopathy","volume":"119","author":"Gavriilaki","year":"2019","journal-title":"Thromb. Haemost."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/j.blre.2019.03.003","article-title":"Hypertension in hematologic malignancies and hematopoietic cell transplantation: An emerging issue with the introduction of novel treatments","volume":"35","author":"Gavriilaki","year":"2019","journal-title":"Blood Rev."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1018","DOI":"10.2146\/ajhp170708","article-title":"Disseminated cryptococcosis associated with administration of eculizumab","volume":"75","author":"Clancy","year":"2018","journal-title":"J. Am. Health Syst. Pharm."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.jinf.2018.10.015","article-title":"Unusual Neisseria species as a cause of infection in patients taking eculizumab","volume":"78","author":"Crew","year":"2019","journal-title":"J. Infect."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1097\/QCO.0000000000000279","article-title":"nfections associated with the use of eculizumab: Recommendations for prevention and prophylaxis","volume":"29","author":"Benamu","year":"2016","journal-title":"Curr. Opin. Infect. Dis."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1111\/bjh.12347","article-title":"Long-term safety and efficacy of sustained eculizumab treatment in patients with paroxysmal nocturnal haemoglobinuria","volume":"162","author":"Hillmen","year":"2013","journal-title":"Br. J. Haematol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1038\/nrd4657","article-title":"Complement, a target for therapy in inflammatory and degenerative diseases","volume":"14","author":"Morgan","year":"2015","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1038\/nrneph.2017.156","article-title":"The renaissance of complement therapeutics","volume":"14","author":"Ricklin","year":"2018","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1053\/j.seminhematol.2018.02.002","article-title":"Expanding Complement Therapeutics for the Treatment of Paroxysmal Nocturnal Hemoglobinuria","volume":"55","author":"Mastellos","year":"2018","journal-title":"Semin. Hematol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"2152","DOI":"10.1172\/JCI136094","article-title":"Complementopathies and precision medicine","volume":"130","author":"Gavriilaki","year":"2020","journal-title":"J. Clin. Investig."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1182\/blood-2018-09-876805","article-title":"Ravulizumab (ALXN1210) vs eculizumab in C5-inhibitor\u2013experienced adult patients with PNH: The 302 study","volume":"133","author":"Kulasekararaj","year":"2019","journal-title":"Blood"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1182\/blood-2018-09-876136","article-title":"Ravulizumab (ALXN1210) vs eculizumab in adult patients with PNH naive to complement inhibitors: The 301 study","volume":"133","author":"Lee","year":"2019","journal-title":"Blood"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"2231","DOI":"10.1182\/blood-2019-128746","article-title":"One-Year Efficacy and Safety from a Phase 3 Trial of Ravulizumab in Adult Patients with Paroxysmal Nocturnal Hemoglobinuria Receiving Prior Eculizumab Treatment","volume":"134","author":"Kulasekararaj","year":"2019","journal-title":"ASH Blood"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"952","DOI":"10.1182\/blood-2019-128929","article-title":"Breakthrough Hemolysis in Adult Patients with Paroxysmal Nocturnal Hemoglobinuria Treated with Ravulizumab: Results of a 52-Week Extension from Two Phase 3 Studies","volume":"134","author":"Hill","year":"2019","journal-title":"Blood"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1016\/j.jneuroim.2016.04.013","article-title":"Acute and prolonged complement activation in the central nervous system during herpes simplex encephalitis","volume":"295","author":"Eriksson","year":"2016","journal-title":"J. Neuroimmunol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.virol.2011.01.003","article-title":"The lectin pathway of complement activation contributes to protection from West Nile virus infection","volume":"412","author":"Fuchs","year":"2011","journal-title":"Virology"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1016\/j.jinf.2013.12.016","article-title":"Cerebrospinal fluid complement activation in patients with pneumococcal and meningococcal meningitis","volume":"68","author":"Brouwer","year":"2014","journal-title":"J. Infect."},{"key":"ref_55","doi-asserted-by":"crossref","unstructured":"Brouwer, M.C., Baas, F., Van Der Ende, A., and Van De Beek, D. (2013). Genetic Variation and Cerebrospinal Fluid Levels of Mannose Binding Lectin in Pneumococcal Meningitis Patients. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0065151"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"3325","DOI":"10.1093\/brain\/awz222","article-title":"Targeting the complement system in bacterial meningitis","volume":"142","author":"Koelman","year":"2019","journal-title":"Brain"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"108450","DOI":"10.1016\/j.clim.2020.108450","article-title":"The first case of COVID-19 treated with the complement C3 inhibitor AMY-101","volume":"215","author":"Mastaglio","year":"2020","journal-title":"Clin. Immunol."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"108555","DOI":"10.1016\/j.clim.2020.108555","article-title":"Anti-complement C5 therapy with eculizumab in three cases of critical COVID-19","volume":"219","author":"Laurence","year":"2020","journal-title":"Clin. Immunol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1001\/jamaneurol.2020.1127","article-title":"Neurologic Manifestations of Hospitalized Patients with Coronavirus Disease 2019 in Wuhan, China","volume":"77","author":"Mao","year":"2020","journal-title":"JAMA Neurol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"e227","DOI":"10.1111\/bjh.16783","article-title":"Severe COVID-19 infection and thrombotic microangiopathy: Success does not come easily","volume":"189","author":"Gavriilaki","year":"2020","journal-title":"Br. J. Haematol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s11906-020-01078-6","article-title":"Endothelial Dysfunction in COVID-19: Lessons Learned from Coronaviruses","volume":"22","author":"Gavriilaki","year":"2020","journal-title":"Curr. Hypertens. Rep."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1186\/s13063-020-04548-z","article-title":"A Phase 3 Open-label, Randomized, Controlled Study to Evaluate the Efficacy and Safety of Intravenously Administered Ravulizumab Compared with Best Supportive Care in Patients with COVID-19 Severe Pneumonia, Acute Lung Injury, or Acute Respiratory Distress Syndrome: A structured summary of a study protocol for a randomised controlled trial","volume":"21","author":"Smith","year":"2020","journal-title":"Trials"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"857","DOI":"10.3389\/fphar.2020.00857","article-title":"Combination of Ruxolitinib and Eculizumab for Treatment of Severe SARS-CoV-2-Related Acute Respiratory Distress Syndrome: A Controlled Study","volume":"11","author":"Giudice","year":"2020","journal-title":"Front. Pharmacol."},{"key":"ref_64","doi-asserted-by":"crossref","unstructured":"Vlaar, A.P.J., de Bruin, S., Busch, M., Timmermans, S.A.M.E.G., van Zeggeren, I.E., Koning, R., Ter Horst, L., Bulle, E.B., Baarle, F.E.H.P.V., and van de Poll, M.C.G. (2020). Anti-C5a antibody IFX-1 (vilobelimab) treatment versus best supportive care for patients with severe COVID-19 (PANAMO): An exploratory, open-label, phase 2 randomised controlled trial. Lancet Rheumatol., in press.","DOI":"10.1016\/S2665-9913(20)30341-6"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1038\/s41572-019-0079-y","article-title":"Myasthenia Gravis","volume":"5","author":"Aarli","year":"2019","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_66","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_67","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1084\/jem.147.4.973","article-title":"Role of complement in the pathogenesis of experimental autoimmune myasthenia gravis","volume":"147","author":"Lennon","year":"1978","journal-title":"J. Exp. Med."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"177","DOI":"10.3181\/00379727-105-26050","article-title":"Changes in Serum Complement Activity in Patients with Myasthenia Gravis","volume":"105","author":"Nastuk","year":"1960","journal-title":"Proc. Soc. Exp. Biol. Med."},{"key":"ref_69","first-page":"78","article-title":"Myasthenia gravis: Demonstration of membrane attack complex in muscle end-plates","volume":"5","author":"Fazekas","year":"1986","journal-title":"Clin. Neuropathol."},{"key":"ref_70","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_71","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1111\/j.1365-2249.2006.03205.x","article-title":"The membrane attack pathway of complement drives pathology in passively induced experimental autoimmune myasthenia gravis in mice","volume":"146","author":"Morgan","year":"2006","journal-title":"Clin. Exp. Immunol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"8562","DOI":"10.4049\/jimmunol.179.12.8562","article-title":"Anti-C5 Antibody Treatment Ameliorates Weakness in Experimentally Acquired Myasthenia Gravis","volume":"179","author":"Zhou","year":"2007","journal-title":"J. Immunol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1002\/ana.21536","article-title":"Novel complement inhibitor limits severity of experimentally myasthenia gravis","volume":"65","author":"Soltys","year":"2009","journal-title":"Ann. Neurol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1016\/j.jneuroim.2006.10.014","article-title":"Pros and cons of treating murine myasthenia gravis with anti-C1q antibody","volume":"182","author":"Tuzun","year":"2007","journal-title":"J. Neuroimmunol."},{"key":"ref_75","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_76","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_77","doi-asserted-by":"crossref","first-page":"2641","DOI":"10.1007\/s11136-019-02214-9","article-title":"Eculizumab improves fatigue in refractory generalized myasthenia gravis","volume":"28","author":"Haraldstad","year":"2019","journal-title":"Qual. Life Res."},{"key":"ref_78","doi-asserted-by":"crossref","unstructured":"Oyama, M., Okada, K., Masuda, M., Shimizu, Y., Yokoyama, K., Uzawa, A., Kawaguchi, N., Ikeguchi, R., Hoshino, Y., and Hatano, T. (2020). Suitable indications of eculizumab for patients with refractory generalized myasthenia gravis. Ther. Adv. Neurol. Disord., 13.","DOI":"10.1177\/1756286420904207"},{"key":"ref_79","unstructured":"Pharmaceuticals, R. (2020, October 24). A Phase 2, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Safety, Tolerability, and Preliminary Efficacy of RA101495 in Subjects with Generalized Myasthenia Gravis, Available online: https:\/\/clinicaltrials.gov\/ct2\/show\/NCT03315130?cond=A+Phase+2%2C+Multicenter%2C+Randomized%2C+Double-Blind%2C+Placebo-Controlled+Study+to+Evaluate+the+Safety%2C+Tolerability%2C+and+Preliminary+Efficacy+of+RA101495+in+Subjects+With+Generalized+Myasthenia+Gravis&draw=2&rank=1."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"1502","DOI":"10.1016\/S0140-6736(08)61620-7","article-title":"Multiple sclerosis","volume":"372","author":"Coles","year":"2008","journal-title":"Lancet"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"942","DOI":"10.1056\/NEJMra052130","article-title":"Multiple Sclerosis\u2014The Plaque and Its Pathogenesis","volume":"354","author":"Frohman","year":"2006","journal-title":"N. Engl. J. Med."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"5840286","DOI":"10.1155\/2019\/5840286","article-title":"Autologous Hematopoietic Cell Transplantation in Multiple Sclerosis: Changing Paradigms in the Era of Novel Agents","volume":"2019","author":"Gavriilaki","year":"2019","journal-title":"Stem Cells Int."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"2106","DOI":"10.1016\/S0140-6736(04)17551-X","article-title":"A serum autoantibody marker of neuromyelitis optica: Distinction from multiple sclerosis","volume":"364","author":"Lennon","year":"2004","journal-title":"Lancet"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1212\/WNL.0b013e318248dec1","article-title":"Serologic diagnosis of NMO: A multicenter comparison of aquaporin-4-IgG assays","volume":"78","author":"Waters","year":"2012","journal-title":"Neurology"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s11940-015-0387-9","article-title":"Treatment of Neuromyelitis Optica Spectrum Disorder: Acute, Preventive, and Symptomatic","volume":"18","author":"Kessler","year":"2016","journal-title":"Curr. Treat. Options Neurol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1212\/WNL.0000000000002366","article-title":"International consensus diagnostic criteria for neuromyelitis optica spectrum disordersAuthor Response","volume":"86","author":"Tan","year":"2016","journal-title":"Neurology"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1002\/ana.24554","article-title":"Neuromyelitis optica: Evaluation of 871 attacks and 1,153 treatment courses","volume":"79","author":"Kleiter","year":"2016","journal-title":"Ann. Neurol."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1016\/S1474-4422(13)70076-0","article-title":"Eculizumab in AQP4-IgG-positive relapsing neuromyelitis optica spectrum disorders: An open-label pilot study","volume":"12","author":"Pittock","year":"2013","journal-title":"Lancet Neurol."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1111\/bpa.12546","article-title":"Tissue microarray methodology identifies complement pathway activation and dysregulation in progressive multiple sclerosis","volume":"28","author":"Loveless","year":"2018","journal-title":"Brain Pathol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"3290","DOI":"10.1073\/pnas.79.10.3290","article-title":"Complement activation by isolated myelin: Activation of the classical pathway in the absence of myelin-specific antibodies","volume":"79","author":"Vanguri","year":"1982","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1111\/j.1365-2990.1989.tb01231.x","article-title":"Immunocytochemical localization of the terminal complement complex in multiple sclerosis","volume":"15","author":"Compston","year":"1989","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/2051-5960-2-53","article-title":"Complement activation in multiple sclerosis plaques: An immunohistochemical analysis","volume":"2","author":"Ingram","year":"2014","journal-title":"Acta Neuropathol. Commun."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1016\/j.bbi.2020.03.004","article-title":"Complement-dependent synapse loss and microgliosis in a mouse model of multiple sclerosis","volume":"87","author":"Hammond","year":"2020","journal-title":"Brain Behav. Immun."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1177\/1352458518760715","article-title":"Convergent effects of a functional C3 variant on brain atrophy, demyelination, and cognitive impairment in multiple sclerosis","volume":"25","author":"Roostaei","year":"2019","journal-title":"Mult. Scler. J."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1001\/jamaneurol.2017.4842","article-title":"Differences in the Reponses to Apheresis Therapy of Patients with 3 Histopathologically Classified Immunopathological Patterns of Multiple Sclerosis","volume":"75","author":"Stork","year":"2018","journal-title":"JAMA Neurol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"577147","DOI":"10.1016\/j.jneuroim.2020.577147","article-title":"Complement activation in cerebrospinal fluid in clinically isolated syndrome and early stages of relapsing remitting multiple sclerosis","volume":"340","author":"Ernerudh","year":"2020","journal-title":"J. Neuroimmunol."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"1103","DOI":"10.1007\/s12026-017-8961-8","article-title":"The complement system as a biomarker of disease activity and response to treatment in multiple sclerosis","volume":"65","author":"Tatomir","year":"2017","journal-title":"Immunol. Res."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s12974-018-1333-z","article-title":"Complement-dependent bystander injury to neurons in AQP4-IgG seropositive neuromyelitis optica","volume":"15","author":"Duan","year":"2018","journal-title":"J. Neuroinflamm."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"2000","DOI":"10.1172\/JCI122942","article-title":"Membrane assembly of aquaporin-4 autoantibodies regulates classical complement activation in neuromyelitis optica","volume":"129","author":"Soltys","year":"2019","journal-title":"J. Clin. Investig."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1007\/s12264-019-00375-8","article-title":"Myelin Oligodendrocyte Glycoprotein-IgG Contributes to Oligodendrocytopathy in the Presence of Complement, Distinct from Astrocytopathy Induced by AQP4-IgG","volume":"35","author":"Fang","year":"2019","journal-title":"Neurosci. Bull."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1186\/s12974-019-1448-x","article-title":"CD55 upregulation in astrocytes by statins as potential therapy for AQP4-IgG seropositive neuromyelitis optica","volume":"16","author":"Tradtrantip","year":"2019","journal-title":"J. Neuroinflamm."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"614","DOI":"10.1056\/NEJMoa1900866","article-title":"Eculizumab in Aquaporin-4\u2013Positive Neuromyelitis Optica Spectrum Disorder","volume":"381","author":"Pittock","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1007\/s40265-018-1039-7","article-title":"Pharmacotherapy for Neuromyelitis Optica Spectrum Disorders: Current Management and Future Options","volume":"79","author":"Collongues","year":"2019","journal-title":"Drugs"},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"2120","DOI":"10.1016\/S0140-6736(08)60921-6","article-title":"Spinal muscular atrophy","volume":"371","author":"Lunn","year":"2008","journal-title":"Lancet"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1007\/s00439-002-0828-x","article-title":"Genetic testing and risk assessment for spinal muscular atrophy (SMA)","volume":"111","author":"Ogino","year":"2002","journal-title":"Hum. Genet."},{"key":"ref_106","unstructured":"EM Agency (2020, October 24). First Medicine for Spinal Muscular Atrophy. Secondary First Medicine for Spinal Muscular Atrophy. Available online: http:\/\/www.ema.europa.eu\/ema\/index.jsp?curl=pages\/news_and_events\/news\/2017\/04\/news_detail_002735.jsp&mid=WC0b01ac058004d5c1."},{"key":"ref_107","unstructured":"U.S. Food and Drug Administration (2020, October 24). FDA Approves Innovative Gene Therapy to Treat Pediatric Patients with Spinal Muscular Atrophy, a Rare Disease and Leading Genetic Cause of Infant Mortality. Secondary FDA Approves Innovative Gene Therapy to Treat Pediatric Patients with Spinal Muscular Atrophy, a Rare Disease and Leading Genetic Cause of Infant Mortality, Available online: https:\/\/www-fda-gov.proxy1.library.jhu.edu\/news-events\/press-announcements\/fda-approves-innovative-gene-therapy-treat-pediatric-patients-spinal-muscular-atrophy-rare-disease."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"19348","DOI":"10.1073\/pnas.1319280110","article-title":"Dysregulation of synaptogenesis genes antecedes motor neuron pathology in spinal muscular atrophy","volume":"110","author":"Zhang","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"3087","DOI":"10.1016\/j.celrep.2019.11.013","article-title":"The Classical Complement Pathway Mediates Microglia-Dependent Remodeling of Spinal Motor Circuits during Development and in SMA","volume":"29","author":"Vukojicic","year":"2019","journal-title":"Cell Rep."},{"key":"ref_110","first-page":"CD001447","article-title":"Riluzole for amyotrophic lateral sclerosis (ALS)\/motor neuron disease (MND)","volume":"3","author":"Miller","year":"2012","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1016\/S1474-4422(17)30115-1","article-title":"Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: A randomised, double-blind, placebo-controlled trial","volume":"16","author":"Writing","year":"2017","journal-title":"Lancet Neurol."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1111\/j.1600-0404.1991.tb04656.x","article-title":"Serum and CSF immunological findings in ALS","volume":"83","author":"Apostolski","year":"1991","journal-title":"Acta Neurol. Scand."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1016\/S0022-510X(97)00225-6","article-title":"Cerebrospinal fluid complement activation in neurological diseases","volume":"154","author":"Brinar","year":"1998","journal-title":"J. Neurol. Sci."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.nbd.2011.01.002","article-title":"Innate and adaptive immunity in amyotrophic lateral sclerosis: Evidence of complement activation","volume":"42","author":"Sta","year":"2011","journal-title":"Neurobiol. Dis."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1186\/s12974-016-0538-2","article-title":"Complement activation at the motor end-plates in amyotrophic lateral sclerosis","volume":"13","author":"Bosch","year":"2016","journal-title":"J. Neuroinflamm."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/0165-5728(84)90042-0","article-title":"Deposits of IgG and C3 in the spinal cord and motor cortex of ALS patients","volume":"6","author":"Donnenfeld","year":"1984","journal-title":"J. Neuroimmunol."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.jneuroim.2014.09.005","article-title":"Elevation of the terminal complement activation products C5a and C5b-9 in ALS patient blood","volume":"276","author":"Mantovani","year":"2014","journal-title":"J. Neuroimmunol."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1186\/s12974-018-1217-2","article-title":"Complement components are upregulated and correlate with disease progression in the TDP-43Q331K mouse model of amyotrophic lateral sclerosis","volume":"15","author":"Lee","year":"2018","journal-title":"J. Neuroinflamm."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"E4385","DOI":"10.1073\/pnas.1318309110","article-title":"C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice","volume":"110","author":"Lobsiger","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"8727","DOI":"10.4049\/jimmunol.181.12.8727","article-title":"The Complement Factor C5a Contributes to Pathology in a Rat Model of Amyotrophic Lateral Sclerosis","volume":"181","author":"Woodruff","year":"2008","journal-title":"J. Immunol."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"1778","DOI":"10.1212\/WNL.0b013e31828726f5","article-title":"Alzheimer disease in the United States (2010\u20132050) estimated using the 2010 census","volume":"80","author":"Hebert","year":"2013","journal-title":"Neurology"},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1016\/j.jalz.2011.05.901","article-title":"Dementia and Alzheimer\u2019s disease: A new direction. The 2010 Jay L. Foster Memorial Lecture","volume":"7","author":"Kuller","year":"2011","journal-title":"Alzheimers Dement."},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"S13","DOI":"10.1093\/ije\/20.Supplement_2.S62","article-title":"Familial Aggregation of Alzheimer\u2019s Disease and Related Disorders: A Collaborative Re-Analysis of Case-Control Studies","volume":"20","author":"Clayton","year":"1991","journal-title":"Int. J. Epidemiol."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"2996","DOI":"10.1089\/scd.2013.0511","article-title":"Induced pluripotent stem cells from familial Alzheimer\u2019s disease patients differentiate into mature neurons with amyloidogenic properties","volume":"23","author":"Mahairaki","year":"2014","journal-title":"Stem Cells Dev."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1007\/BF00687336","article-title":"Immuno-electron-microscopic localization of complements in amyloid fibrils of senile plaques","volume":"63","author":"Ishii","year":"1984","journal-title":"Acta Neuropathol."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1016\/S0197-4580(97)00042-0","article-title":"Molecular and Cellular Characterization of the Membrane Attack Complex, C5b-9, in Alzheimer\u2019s Disease","volume":"18","author":"Webster","year":"1997","journal-title":"Neurobiol. Aging"},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1016\/j.cell.2015.12.056","article-title":"The Cellular Phase of Alzheimer\u2019s Disease","volume":"164","author":"Karran","year":"2016","journal-title":"Cell"},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"6017","DOI":"10.1007\/s12035-019-1479-4","article-title":"Upregulation of Proteolytic Pathways and Altered Protein Biosynthesis Underlie Retinal Pathology in a Mouse Model of Alzheimer\u2019s Disease","volume":"56","author":"Mirzaei","year":"2019","journal-title":"Mol. Neurobiol."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1523\/JNEUROSCI.2117-15.2016","article-title":"Astrocyte-Microglia Cross Talk through Complement Activation Modulates Amyloid Pathology in Mouse Models of Alzheimer\u2019s Disease","volume":"36","author":"Lian","year":"2016","journal-title":"J. Neurosci."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"1322","DOI":"10.1016\/j.neuron.2018.10.014","article-title":"Changes in the Synaptic Proteome in Tauopathy and Rescue of Tau-Induced Synapse Loss by C1q Antibodies","volume":"100","author":"Dejanovic","year":"2018","journal-title":"Neuron"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1177\/1091581817740873","article-title":"Nonclinical Development of ANX005: A Humanized Anti-C1q Antibody for Treatment of Autoimmune and Neurodegenerative Diseases","volume":"36","author":"Lansita","year":"2017","journal-title":"Int. J. Toxicol."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.4049\/jimmunol.0901005","article-title":"Treatment with a C5aR Antagonist Decreases Pathology and Enhances Behavioral Performance in Murine Models of Alzheimer\u2019s Disease","volume":"183","author":"Fonseca","year":"2009","journal-title":"J. Immunol."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"2080","DOI":"10.1111\/j.1471-4159.2008.05558.x","article-title":"Complement C3 and C4 expression in C1q sufficient and deficient mouse models of Alzheimer\u2019s disease","volume":"106","author":"Zhou","year":"2008","journal-title":"J. Neurochem."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"10837","DOI":"10.1073\/pnas.162350199","article-title":"Prominent neurodegeneration and increased plaque formation in complement-inhibited Alzheimer\u2019s mice","volume":"99","author":"Yan","year":"2002","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"1452","DOI":"10.1038\/ng.2802","article-title":"Meta-analysis of 74,046 individuals identifies 11 new susceptibility loci for Alzheimer\u2019s disease","volume":"45","author":"Lambert","year":"2013","journal-title":"Nat. Genet."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1016\/j.cell.2013.03.030","article-title":"Integrated Systems Approach Identifies Genetic Nodes and Networks in Late-Onset Alzheimer\u2019s Disease","volume":"153","author":"Zhang","year":"2013","journal-title":"Cell"},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"707","DOI":"10.3233\/JAD-160420","article-title":"Complement Biomarkers as Predictors of Disease Progression in Alzheimer\u2019s Disease","volume":"54","author":"Hakobyan","year":"2016","journal-title":"J. Alzheimers Dis."},{"key":"ref_138","first-page":"61","article-title":"Complement protein levels in plasma astrocyte-derived exosomes are abnormal in conversion from mild cognitive impairment to Alzheimer\u2019s disease dementia","volume":"11","author":"Winston","year":"2019","journal-title":"Alzheimers Dement."},{"key":"ref_139","doi-asserted-by":"crossref","unstructured":"Nogueras-Ortiz, C.J., Mahairaki, V., Delgado-Peraza, F., Das, D., Avgerinos, K., Eren, E., Hentschel, M., Goetzl, E.J., Mattson, M.P., and Kapogiannis, D. (2020). Astrocyte- and Neuron-Derived Extracellular Vesicles from Alzheimer\u2019s Disease Patients Effect Complement-Mediated Neurotoxicity. Cells, 9.","DOI":"10.1101\/2020.04.14.041863"},{"key":"ref_140","first-page":"9","article-title":"The discovery of dopamine deficiency in the parkinsonian brain","volume":"2006","author":"Hornykiewicz","year":"2006","journal-title":"J. Neural Transm. Suppl."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1007\/s00335-011-9330-x","article-title":"Parkinson\u2019s disease mouse models in translational research","volume":"22","author":"Antony","year":"2011","journal-title":"Mamm. Genome"},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"1096","DOI":"10.1016\/j.bbrc.2007.04.055","article-title":"Complement activation by islet amyloid polypeptide (IAPP) and \u03b1-synuclein 112","volume":"357","author":"Klegeris","year":"2007","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1007\/BF00427222","article-title":"Lewy bodies in Parkinson\u2019s disease are recognized by antibodies to complement proteins","volume":"84","author":"Yamada","year":"1992","journal-title":"Acta Neuropathol."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1186\/1742-2094-3-29","article-title":"Complement activation in the Parkinson\u2019s disease substantia nigra: An immunocytochemical study","volume":"3","author":"Loeffler","year":"2006","journal-title":"J. Neuroinflamm."},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1007\/s004010000225","article-title":"Activated microglial cells and complement factors are unrelated to cortical Lewy bodies","volume":"100","author":"Rozemuller","year":"2000","journal-title":"Acta Neuropathol."},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1097\/NEN.0b013e31820805b9","article-title":"Possible Involvement of Complement Factor C1q in the Clearance of Extracellular Neuromelanin from the Substantia Nigra in Parkinson Disease","volume":"70","author":"Depboylu","year":"2011","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"1034","DOI":"10.1016\/j.bbrc.2006.02.051","article-title":"Complement C3c and related protein biomarkers in amyotrophic lateral sclerosis and Parkinson\u2019s disease","volume":"342","author":"Goldknopf","year":"2006","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"1509","DOI":"10.1016\/j.ajpath.2011.01.006","article-title":"Complement 3 and Factor H in Human Cerebrospinal Fluid in Parkinson\u2019s Disease, Alzheimer\u2019s Disease, and Multiple-System Atrophy","volume":"178","author":"Wang","year":"2011","journal-title":"Am. J. Pathol."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.neuroscience.2018.09.017","article-title":"Proteomic Profiling of Exosomal Proteins for Blood-based Biomarkers in Parkinson\u2019s Disease","volume":"392","author":"Kitamura","year":"2018","journal-title":"Neuroscience"},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"e17478","DOI":"10.1097\/MD.0000000000017478","article-title":"Differential proteomic analysis of serum exosomes reveals alterations in progression of Parkinson disease","volume":"98","author":"Jiang","year":"2019","journal-title":"Medicine"},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1016\/j.brainres.2007.08.033","article-title":"Complement 3-deficient mice are not protected against MPTP-induced dopaminergic neurotoxicity","volume":"1178","author":"Liang","year":"2007","journal-title":"Brain Res."},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/j.jneuroim.2011.05.006","article-title":"Upregulation of microglial C1q expression has no effects on nigrostriatal dopaminergic injury in the MPTP mouse model of Parkinson disease","volume":"236","author":"Depboylu","year":"2011","journal-title":"J. Neuroimmunol."},{"key":"ref_153","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1016\/j.redox.2017.09.017","article-title":"Complement receptor 3 mediates NADPH oxidase activation and dopaminergic neurodegeneration through a Src-Erk-dependent pathway","volume":"14","author":"Hou","year":"2018","journal-title":"Redox Biol."},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"15005","DOI":"10.1038\/nrdp.2015.5","article-title":"Huntington\u2019s disease","volume":"1","author":"Gusella","year":"2015","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","article-title":"Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain","volume":"277","author":"DiFiglia","year":"1997","journal-title":"Science"},{"key":"ref_156","first-page":"CD006456","article-title":"Therapeutic interventions for symptomatic treatment in Huntington\u2019s disease","volume":"3","author":"Mestre","year":"2009","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1006\/exnr.1999.7170","article-title":"Increased Complement Biosynthesis by Microglia and Complement Activation on Neurons in Huntington\u2019s Disease","volume":"159","author":"Singhrao","year":"1999","journal-title":"Exp. Neurol."},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"1638","DOI":"10.1212\/01.wnl.0000222734.56412.17","article-title":"Microglial activation correlates with severity in Huntington disease: A clinical and PET study","volume":"66","author":"Pavese","year":"2006","journal-title":"Neurology"},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"1759","DOI":"10.1093\/brain\/awm044","article-title":"Microglial activation in presymptomatic Huntington\u2019s disease gene carriers","volume":"130","author":"Tai","year":"2007","journal-title":"Brain"},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"4737","DOI":"10.1172\/JCI64484","article-title":"Mutant huntingtin impairs immune cell migration in Huntington disease","volume":"122","author":"Kwan","year":"2012","journal-title":"J. Clin. Investig."},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"2833","DOI":"10.1021\/pr0700753","article-title":"Proteomic Profiling of Plasma in Huntington\u2019s Disease Reveals Neuroinflammatory Activation and Biomarker Candidates","volume":"6","author":"Dalrymple","year":"2007","journal-title":"J. Proteome Res."},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1096\/fj.05-5814com","article-title":"Therapeutic activity of C5a receptor antagonists in a rat model of neurodegeneration","volume":"20","author":"Woodruff","year":"2006","journal-title":"FASEB J."},{"key":"ref_163","doi-asserted-by":"crossref","first-page":"107","DOI":"10.3233\/JHD-2012-120021","article-title":"Genetic Deficiency of Complement Component 3 Does Not Alter Disease Progression in a Mouse Model of Huntington\u2019s Disease","volume":"1","author":"Larkin","year":"2012","journal-title":"J. Huntingt. Dis."},{"key":"ref_164","doi-asserted-by":"crossref","first-page":"1209","DOI":"10.1016\/j.imbio.2016.06.186","article-title":"A pathogenic role for the C5a receptor, C5aR2, in mouse models of Huntington\u2019s and Parkinson\u2019s disease","volume":"221","author":"Li","year":"2016","journal-title":"Immunobiology"},{"key":"ref_165","doi-asserted-by":"crossref","first-page":"S21","DOI":"10.1002\/ana.410270707","article-title":"Assessment of current diagnostic criteria for Guillain-Barre syndrome","volume":"27","author":"Asbury","year":"1990","journal-title":"Ann. Neurol."},{"key":"ref_166","first-page":"621","article-title":"Chronic inflammatory polyradiculoneuropathy","volume":"50","author":"Dyck","year":"1975","journal-title":"Mayo Clin. Proc."},{"key":"ref_167","first-page":"CD008630","article-title":"Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barr\u00e9 syndrome","volume":"11","author":"Pritchard","year":"2016","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_168","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1212\/WNL.61.6.736","article-title":"Practice parameter: Immunotherapy for Guillain-Barr\u00e9 syndrome: Report of the Quality Standards Subcommittee of the American Academy of Neurology","volume":"61","author":"Hughes","year":"2003","journal-title":"Neurology"},{"key":"ref_169","doi-asserted-by":"crossref","unstructured":"(1985). Plasmapheresis and acute Guillain-Barr\u00e9 syndrome. The Guillain-Barr\u00e9 syndrome Study Group. Neurology, 35, 1096\u20131104.","DOI":"10.1212\/WNL.35.8.1096"},{"key":"ref_170","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1002\/ana.410390512","article-title":"Immune attack on the schwann cell surface in acute inflammatory demyelinating polyneuropathy","volume":"39","author":"Sheikh","year":"1996","journal-title":"Ann. Neurol."},{"key":"ref_171","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1212\/WNL.37.6.1006","article-title":"Guillain-barre syndrome: Activated complement components C3a and C5a in CSF","volume":"37","author":"Hartung","year":"1987","journal-title":"Neurology"},{"key":"ref_172","doi-asserted-by":"crossref","first-page":"3956","DOI":"10.1523\/JNEUROSCI.4401-06.2007","article-title":"Anti-GM1 Antibodies Cause Complement-Mediated Disruption of Sodium Channel Clusters in Peripheral Motor Nerve Fibers","volume":"27","author":"Susuki","year":"2007","journal-title":"J. Neurosci."},{"key":"ref_173","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1136\/jnnp-2011-300309","article-title":"Antiganglioside antibodies are associated with axonal Guillain\u2013Barr\u00e9 syndrome: A Japanese\u2013Italian collaborative study","volume":"83","author":"Sekiguchi","year":"2012","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_174","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1002\/acn3.331","article-title":"Terminal complement activation is increased and associated with disease severity in CIDP","volume":"3","author":"Quast","year":"2016","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_175","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/j.jneuroim.2019.02.001","article-title":"IVIG efficacy in CIDP patients is not associated with terminal complement inhibition","volume":"330","author":"Keller","year":"2019","journal-title":"J. Neuroimmunol."},{"key":"ref_176","doi-asserted-by":"crossref","first-page":"1197","DOI":"10.1093\/brain\/awm316","article-title":"Eculizumab prevents anti-ganglioside antibody-mediated neuropathy in a murine model","volume":"131","author":"Halstead","year":"2008","journal-title":"Brain"},{"key":"ref_177","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1111\/jns.12194","article-title":"Inhibition of complement in Guillain-Barr\u00e9 syndrome: The ICA-GBS study","volume":"22","author":"Davidson","year":"2017","journal-title":"J. Peripher. Nerv. Syst."},{"key":"ref_178","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1016\/S1474-4422(18)30114-5","article-title":"Safety and efficacy of eculizumab in Guillain-Barr\u00e9 syndrome: A multicentre, double-blind, randomised phase 2 trial","volume":"17","author":"Misawa","year":"2018","journal-title":"Lancet Neurol."},{"key":"ref_179","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1016\/j.mpmed.2012.06.005","article-title":"Stroke: Causes and clinical features","volume":"40","author":"Markus","year":"2012","journal-title":"Medicine"},{"key":"ref_180","doi-asserted-by":"crossref","first-page":"e344","DOI":"10.1161\/STR.0000000000000211","article-title":"Guidelines for the Early Management of Patients with Acute Ischemic Stroke: 2019 Update to the 2018 Guidelines for the Early Management of Acute Ischemic Stroke: A Guideline for Healthcare Professionals from the American Heart Association\/American Stroke Association","volume":"50","author":"Powers","year":"2019","journal-title":"Stroke"},{"key":"ref_181","unstructured":"Global Burden of Disease (2016). Global, regional, and national disability-adjusted life-years (DALYs) for 315 diseases and injuries and healthy life expectancy (HALE), 1990\u20132015: A systematic analysis for the Global Burden of Disease Study 2015. Lancet, 388, 1603\u20131658."},{"key":"ref_182","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s12974-015-0464-8","article-title":"Modulation of post-stroke degenerative and regenerative processes and subacute protection by site-targeted inhibition of the alternative pathway of complement","volume":"12","author":"Alawieh","year":"2015","journal-title":"J. Neuroinflamm."},{"key":"ref_183","doi-asserted-by":"crossref","first-page":"2244","DOI":"10.1161\/01.STR.0000182237.20807.d0","article-title":"C1q-Deficiency Is Neuroprotective Against Hypoxic-Ischemic Brain Injury in Neonatal Mice","volume":"36","author":"Ten","year":"2005","journal-title":"Stroke"},{"key":"ref_184","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1161\/01.RES.0000232544.90675.42","article-title":"Complement Component C3 Mediates Inflammatory Injury Following Focal Cerebral Ischemia","volume":"99","author":"Mocco","year":"2006","journal-title":"Circ. Res."},{"key":"ref_185","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1227\/01.NEU.0000219221.14280.65","article-title":"Alterations in Plasma Complement Levels Following Human Ischemic Stroke","volume":"59","author":"Mocco","year":"2006","journal-title":"Neurosurgery"},{"key":"ref_186","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s12974-016-0485-y","article-title":"Complement component C3a plays a critical role in endothelial activation and leukocyte recruitment into the brain","volume":"13","author":"Wu","year":"2016","journal-title":"J. Neuroinflamm."},{"key":"ref_187","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1006\/exnr.1999.7273","article-title":"Expression of Receptors for Complement Anaphylatoxins C3a and C5a Following Permanent Focal Cerebral Ischemia in the Mouse","volume":"161","author":"Bernaudin","year":"2000","journal-title":"Exp. Neurol."},{"key":"ref_188","doi-asserted-by":"crossref","first-page":"3680","DOI":"10.1096\/fj.11-202382","article-title":"Generation of complement component C5a by ischemic neurons promotes neuronal apoptosis","volume":"26","author":"Pavlovski","year":"2012","journal-title":"FASEB J."},{"key":"ref_189","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1097\/00001756-200102120-00022","article-title":"Complement anaphylatoxin C3a is selectively protective against NMDA-induced neuronal cell death","volume":"12","author":"Nicole","year":"2001","journal-title":"NeuroReport"},{"key":"ref_190","doi-asserted-by":"crossref","unstructured":"Cervera, A., Planas, A.M., Justicia, C., Urra, X., Jensenius, J.C., Torres, F., Lozano, F., and Chamorro, A. (2010). Genetically-Defined Deficiency of Mannose-Binding Lectin Is Associated with Protection after Experimental Stroke in Mice and Outcome in Human Stroke. PLoS ONE, 5.","DOI":"10.1371\/journal.pone.0008433"},{"key":"ref_191","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1111\/j.1365-2249.2004.02489.x","article-title":"Systemic complement activation following human acute ischaemic stroke","volume":"137","author":"Pedersen","year":"2004","journal-title":"Clin. Exp. Immunol."},{"key":"ref_192","first-page":"e295","article-title":"Multiple biomarkers covering distinct pathways for predicting outcomes after ischemic stroke","volume":"92","author":"Zhong","year":"2018","journal-title":"Neurology"},{"key":"ref_193","doi-asserted-by":"crossref","first-page":"1016","DOI":"10.3171\/jns.2000.92.6.1016","article-title":"Complement activation in the brain after experimental intracerebral hemorrhage","volume":"92","author":"Hua","year":"2000","journal-title":"J. Neurosurg."},{"key":"ref_194","doi-asserted-by":"crossref","first-page":"741","DOI":"10.3171\/jns.1989.71.5.0741","article-title":"Activated complement components C3a and C4a in cerebrospinal fluid and plasma following subarachnoid hemorrhage","volume":"71","author":"Kasuya","year":"1989","journal-title":"J. Neurosurg."},{"key":"ref_195","first-page":"678","article-title":"Complement C5 Contributes to Brain Injury After Subarachnoid Hemorrhage","volume":"11","author":"Meijers","year":"2019","journal-title":"Transl. Stroke Res."},{"key":"ref_196","doi-asserted-by":"crossref","first-page":"eaao6459","DOI":"10.1126\/scitranslmed.aao6459","article-title":"Targeted complement inhibition salvages stressed neurons and inhibits neuroinflammation after stroke in mice","volume":"10","author":"Alawieh","year":"2018","journal-title":"Sci. Transl. Med."},{"key":"ref_197","doi-asserted-by":"crossref","unstructured":"Ducruet, A.F., Zacharia, B.E., Sosunov, S.A., Gigante, P.R., Yeh, M.L., Gorski, J.W., Otten, M.L., Hwang, R.Y., DeRosa, P.A., and Hickman, Z. (2012). Complement Inhibition Promotes Endogenous Neurogenesis and Sustained Anti-Inflammatory Neuroprotection following Reperfused Stroke. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0038664"},{"key":"ref_198","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1227\/01.NEU.0000335079.70222.8D","article-title":"Protective effect of C5a receptor inhibition after murine reperfused stroke","volume":"63","author":"Kim","year":"2008","journal-title":"Neurosurgery"},{"key":"ref_199","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1007\/s12017-017-8474-6","article-title":"Combination Therapy with Low-Dose IVIG and a C1-esterase Inhibitor Ameliorates Brain Damage and Functional Deficits in Experimental Ischemic Stroke","volume":"20","author":"Chen","year":"2018","journal-title":"Neuromol. Med."},{"key":"ref_200","doi-asserted-by":"crossref","first-page":"4042","DOI":"10.1523\/JNEUROSCI.2462-19.2020","article-title":"Complement-Dependent Synaptic Uptake and Cognitive Decline after Stroke and Reperfusion Therapy","volume":"40","author":"Alawieh","year":"2020","journal-title":"J. Neurosci."},{"key":"ref_201","doi-asserted-by":"crossref","first-page":"1484","DOI":"10.1161\/CIRCULATIONAHA.112.103051","article-title":"Targeting Mannose-Binding Lectin Confers Long-Lasting Protection with a Surprisingly Wide Therapeutic Window in Cerebral Ischemia","volume":"126","author":"Orsini","year":"2012","journal-title":"Circulation"},{"key":"ref_202","doi-asserted-by":"crossref","first-page":"4640","DOI":"10.4049\/jimmunol.1201904","article-title":"The Alternative Complement Pathway Propagates Inflammation and Injury in Murine Ischemic Stroke","volume":"189","author":"Elvington","year":"2012","journal-title":"J. Immunol."},{"key":"ref_203","first-page":"98","article-title":"C3a Receptor Antagonist Attenuates Brain Injury after Intracerebral Hemorrhage","volume":"29","author":"Rynkowski","year":"2009","journal-title":"Br. J. Pharmacol."},{"key":"ref_204","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/j.brainres.2009.04.047","article-title":"Synergistic neuroprotective effects of C3a and C5a receptor blockade following intracerebral hemorrhage","volume":"1298","author":"Garrett","year":"2009","journal-title":"Brain Res."},{"key":"ref_205","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1111\/epi.12550","article-title":"ILAE Official Report: A practical clinical definition of epilepsy","volume":"55","author":"Fisher","year":"2014","journal-title":"Epilepsia"},{"key":"ref_206","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.seizure.2018.05.016","article-title":"Complement system biomarkers in epilepsy","volume":"60","author":"Kopczynska","year":"2018","journal-title":"Seizure"},{"key":"ref_207","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1007\/s00429-013-0528-4","article-title":"MRI changes and complement activation correlate with epileptogenicity in a mouse model of temporal lobe epilepsy","volume":"219","author":"Kharatishvili","year":"2014","journal-title":"Brain Struct. Funct."},{"key":"ref_208","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1016\/0192-0561(94)90087-6","article-title":"Humoral and cellular immune parameters in untreated and phenytoin- or carbamazepine-treated epileptic patients","volume":"16","author":"Hincal","year":"1994","journal-title":"Int. J. Immunopharmacol."},{"key":"ref_209","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1523\/JNEUROSCI.23-03-00955.2003","article-title":"Formation of Complement Membrane Attack Complex in Mammalian Cerebral Cortex Evokes Seizures and Neurodegeneration","volume":"23","author":"Xiong","year":"2003","journal-title":"J. Neurosci."},{"key":"ref_210","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1016\/j.nbd.2007.01.015","article-title":"Complement activation in experimental and human temporal lobe epilepsy","volume":"26","author":"Aronica","year":"2007","journal-title":"Neurobiol. Dis."},{"key":"ref_211","doi-asserted-by":"crossref","unstructured":"Jamali, S., Salzmann, A., Perroud, N., Ponsole-Lenfant, M., Cillario, J., Roll, P., Roeckel-Trevisiol, N., Crespel, A., Balzar, J., and Schlachter, K. (2010). Functional Variant in Complement C3 Gene Promoter and Genetic Susceptibility to Temporal Lobe Epilepsy and Febrile Seizures. PLoS ONE, 5.","DOI":"10.1371\/journal.pone.0012740"},{"key":"ref_212","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/j.eplepsyres.2017.09.005","article-title":"Complement system dysregulation in patients affected by Idiopathic Generalized Epilepsy and the effect of antiepileptic treatment","volume":"137","author":"Liguori","year":"2017","journal-title":"Epilepsy Res."},{"key":"ref_213","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1016\/j.expneurol.2017.06.009","article-title":"Enhanced classical complement pathway activation and altered phagocytosis signaling molecules in human epilepsy","volume":"295","author":"Wyatt","year":"2017","journal-title":"Exp. Neurol."},{"key":"ref_214","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.nbd.2017.10.012","article-title":"Status epilepticus triggers long-lasting activation of complement C1q-C3 signaling in the hippocampus that correlates with seizure frequency in experimental epilepsy","volume":"109","author":"Schartz","year":"2018","journal-title":"Neurobiol. Dis."},{"key":"ref_215","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.nbd.2015.02.004","article-title":"A novel anticonvulsant mechanism via inhibition of complement receptor C5ar1 in murine epilepsy models","volume":"76","author":"Benson","year":"2015","journal-title":"Neurobiol. Dis."},{"key":"ref_216","doi-asserted-by":"crossref","first-page":"112705","DOI":"10.1016\/j.physbeh.2019.112705","article-title":"Early treatment with C1 esterase inhibitor improves weight but not memory deficits in a rat model of status epilepticus","volume":"212","author":"Schartz","year":"2019","journal-title":"Physiol. Behav."},{"key":"ref_217","first-page":"e433","article-title":"Exploratory proteomic analysis implicates the alternative complement cascade in primary CNS vasculitis","volume":"93","author":"Retallack","year":"2019","journal-title":"Neurology"},{"key":"ref_218","doi-asserted-by":"crossref","first-page":"1609","DOI":"10.1016\/j.cell.2019.11.010","article-title":"Cross-Species Single-Cell Analysis Reveals Divergence of the Primate Microglia Program","volume":"179","author":"Geirsdottir","year":"2019","journal-title":"Cell"}],"container-title":["Pharmaceuticals"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1424-8247\/13\/11\/341\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:28:09Z","timestamp":1760178489000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1424-8247\/13\/11\/341"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10,26]]},"references-count":218,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2020,11]]}},"alternative-id":["ph13110341"],"URL":"https:\/\/doi.org\/10.3390\/ph13110341","relation":{},"ISSN":["1424-8247"],"issn-type":[{"value":"1424-8247","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,10,26]]}}}