ARGX-119 is a first-in-class humanized agonist monoclonal antibody specific for muscle-specific kinase ( MuSK ). ARGX-119 can be used for the study of neuromuscular diseases. The isotype control for ARGX-119 can refer to Human IgG1 lambda1, Isotype Control [1].
In Vivo, In a mouse model of DOK7 congenital myasthenia (CM), ARGX-119 (20 mg/kg) prevents early postnatal lethality and reverses disease relapse in adult Dok7 CM mice by restoring neuromuscular function and reducing muscle weakness and fatigability in a dose-dependent manner[1].
In Vitro, ARGX-119 binds with high affinity to the Frizzled-like domain of human, nonhuman primate, rat, and mouse MuSK, without off-target binding, making it suitable for clinical development. Within the Fc region, ARGX-119 harbors L234A and L235A mutations to diminish potential immune-activating effector functions. Its mode of action is to activate MuSK, without interfering with its natural ligand neural Agrin, and cluster acetylcholine receptors in a dose-dependent manner, thereby stabilizing neuromuscular function[1].
References:
[1]. Vanhauwaert R, et al. ARGX-119 is an agonist antibody for human MuSK that reverses disease relapse in a mouse model of congenital myasthenic syndrome. Sci Transl Med. 2024 Sep 18;16(765):eado7189.
In Vivo, In a mouse model of DOK7 congenital myasthenia (CM), ARGX-119 (20 mg/kg) prevents early postnatal lethality and reverses disease relapse in adult Dok7 CM mice by restoring neuromuscular function and reducing muscle weakness and fatigability in a dose-dependent manner[1].
In Vitro, ARGX-119 binds with high affinity to the Frizzled-like domain of human, nonhuman primate, rat, and mouse MuSK, without off-target binding, making it suitable for clinical development. Within the Fc region, ARGX-119 harbors L234A and L235A mutations to diminish potential immune-activating effector functions. Its mode of action is to activate MuSK, without interfering with its natural ligand neural Agrin, and cluster acetylcholine receptors in a dose-dependent manner, thereby stabilizing neuromuscular function[1].
References:
[1]. Vanhauwaert R, et al. ARGX-119 is an agonist antibody for human MuSK that reverses disease relapse in a mouse model of congenital myasthenic syndrome. Sci Transl Med. 2024 Sep 18;16(765):eado7189.