ArticleNeurology(R) neuroimmunology & neuroinflammation2022
Anti-C2 Antibody ARGX-117 Inhibits Complement in a Disease Model for Multifocal Motor Neuropathy.
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Article in Neurology(R) neuroimmunology & neuroinflammation, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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14citing papers in PubMed
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Who cites it
14 citing papers in PubMed, 24 citations in OpenAlex.
- Anti-C2-mediated complement inhibition prevents disease in a rat passive transfer model of myasthenia gravis.Journal of translational autoimmunity · 2026Article
- Role of complement and complement-targeted therapeutics in neurological diseases.Nature reviews. Neurology · 2026Review
- In Vivo Efficacy of an Inhibitor of Complement and FcRn in Models of Glomerulonephritis and Collagen-Induced Arthritis Using Human C2 Knock-In Mice.International journal of molecular sciences · 2026Article
- IgM Anti-Ganglioside Binding and Complement Activation in an iPSC-Derived Motor Neuron Model for Multifocal Motor Neuropathy.Neurology(R) neuroimmunology & neuroinflammation · 2026Article
- The changing landscape of primary autoimmune neuropathies.Nature reviews. Neurology · 2025Review
- Systemic lupus erythematosus as the paradigm for understanding the complex immune relationships and therapeutic opportunities for targeting complement in autoimmune diseases.Immunobiology · 2025Review
- Knowing the enemy: strategic targeting of complement to treat Alzheimer disease.Nature reviews. Neurology · 2025Review
- Multifocal Motor Neuropathy: A Narrative Review.Muscle & nerve · 2025Review
- Complement activation in immunological neurological disorders: mechanisms and therapeutic strategies.Frontiers in neurology · 2025Review
- Complement activation by IgM autoantibodies linked to immune-mediated neuropathies depends on C2.European journal of neurology · 2025Article
- IgM anti-GM2 antibodies in patients with multifocal motor neuropathy target Schwann cells and are associated with early onset.Journal of neuroinflammation · 2024Article
- Molecular, Electrophysiological, and Ultrasonographic Differences in Selected Immune-Mediated Neuropathies with Therapeutic Implications.International journal of molecular sciences · 2023Review
- Immune-Mediated Neuropathies: Pathophysiology and Management.International journal of molecular sciences · 2023Review
- Therapeutic Monoclonal Antibody Therapies in Chronic Autoimmune Demyelinating Neuropathies.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2022Review
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Authors and funding
19 authors at 1 institution in 1 country.
Kevin BuddingFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
Lill Eva JohansenFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.ORCID 0000-0003-2187-6709
Inge Van de WalleFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.ORCID 0000-0002-7631-399X
Kim DijkxhoornFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
Elisabeth de ZeeuwFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
Lauri M BloemenkampFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
Jeroen W BosFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
Marc D JansenFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
Chantall A D CurialFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
Karen SilenceFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
Hans de HaardFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.ORCID 0000-0001-8707-0084
Christophe BlanchetotFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.ORCID 0000-0001-9152-9978
Liesbeth Van de VenFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
Jeanette H W LeusenFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
R Jeroen PasterkampFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.ORCID 0000-0003-1631-6440
Leonard H van den BergFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
C Erik HackFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
Peter BorossFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands.
W Ludo van der PolFrom the Center for Translational Immunology (K.B., K.D., E.Z., L.M.B., J.H.W.L., C.E.H., P.B.), University Medical Center Utrecht; Department of Neurology and Neurosurgery (L.E.J., L.M.B., J.W.B., M.D.J., C.A.D.C., L.H.B., W.L.P.), University Medical Center Utrecht Brain Center; Department of Translational Neuroscience (L.E.J., L.M.B., R.J.P.), University Medical Center Utrecht Brain Center, Utrecht University; Argenx BVBA, Industriepark-Zwijnaarde 7 (I.W., K.S., H.H., C.B., L.V.), Zwijnaarde, Belgium; and Prothix (C.E.H., P.B.), Leiden, the Netherlands. w.l.vanderpol@umcutrecht.nl.
University Medical Center Utrecht · NL
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesTo determine the role of complement in the disease pathology of multifocal motor neuropathy (MMN), we investigated complement activation, and inhibition, on binding of MMN patient-derived immunoglobulin M (IgM) antibodies in an induced pluripotent stem cell (iPSC)-derived motor neuron (MN) model for MMN.
methodsiPSC-derived MNs were characterized for the expression of complement receptors and membrane-bound regulators, for the binding of circulating IgM anti-GM1 from patients with MMN, and for subsequent fixation of C4 and C3 on incubation with fresh serum. The potency of ARGX-117, a novel inhibitory monoclonal antibody targeting C2, to inhibit fixation of complement was assessed.
resultsiPSC-derived MNs moderately express the complement regulatory proteins CD46 and CD55 and strongly expressed CD59. Furthermore, MNs express C3aR, C5aR, and complement receptor 1. IgM anti-GM1 antibodies in serum from patients with MMN bind to MNs and induce C3 and C4 fixation on incubation with fresh serum. ARGX-117 inhibits complement activation downstream of C4 induced by patient-derived anti-GM1 antibodies bound to MNs. DISCUSSION: Binding of IgM antibodies from patients with MMN to iPSC-derived MNs induces complement activation. By expressing complement regulatory proteins, particularly CD59, MNs are protected against complement-mediated lysis. Yet, because of expressing C3aR, the function of these cells may be affected by complement activation upstream of membrane attack complex formation. ARGX-117 inhibits complement activation upstream of C3 in this disease model for MMN and therefore represents an intervention strategy to prevent harmful effects of complement in MMN.
Indexed as
Motor NeuronsAntibodies, Monoclonal, HumanizedCells, CulturedComplement ActivationComplement C2HumansImmunoglobulin MInduced Pluripotent Stem CellsPolyneuropathiesAntibodies, Monoclonal, HumanizedComplement C2Immunoglobulin M
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