Anti-C2 Antibody ARGX-117 Inhibits Complement in a Disease Model for Multifocal Motor Neuropathy.
Kevin BuddingLill Eva JohansenInge Van De WalleKim DijkxhoornElisabeth de ZeeuwLauri M BloemenkampJeroen W BosMarc D JansenChantall A D CurialKaren SilenceHans de HaardChristophe BlanchetotLiesbeth Van de VenJeanette H W LeusenR Jeroen PasterkampLeonard H van den BergC Erik HackPeter BorossW Ludo van der PolPublished in: Neurology(R) neuroimmunology & neuroinflammation (2021)
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.