Utility of experimental animal models of myasthenia gravis for the elucidation of pathogenic mechanisms and development of new medications

Myasthenia gravis is an autoimmune disease caused by the association of pathogenic autoantibodies with postsynaptic membrane proteins at neuromuscular junctions. Establishment of the pathogenicity of candidate antibodies with experimental animal models of myasthenia gravis is indispensable. Here, we review the contributions of animal myasthenia gravis models to the elucidation of the pathogenic mechanisms of myasthenia gravis and the development of medications for the disease. AbstractMyasthenia gravis (MG) is an autoimmune disease characterized by ptosis, fatigue and muscle weakness, caused by the association of pathogenic autoantibodies with postsynaptic membrane proteins at neuromuscular junctions. Although various autoantibodies have been found in sera from patients with MG, causal relationships with MG have not been shown for most antibodies. Establishment of the pathogenicity of candidate antibodies with experimental animal models of myasthenia gravis is indispensable. The pathogenicity of autoantibodies against acetylcholine receptors and muscle ‐specific kinase has been confirmed by active immunization with purified antigens or by passive transfer of autoantibodies from patients with MG. Furthermore, animal models of MG have shown not only the pathogenic mechanisms underlying the manifestation of muscle weakness and atrophy observed in p atients with MG, but also the efficacy of novel therapies and the adverse effects of therapeutic drugs. Here, we review the contri...

Source: Clinical and Experimental Neuroimmunology - March 22, 2019 Category: Neurology Authors: Shuuichi Mori, Kazuhiro Shigemoto Tags: Review Article Source Type: research