[Pubmed] Animal models of myasthenia gravis

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Int Rev Neurobiol. 2025;182:145-160. doi: 10.1016/bs.irn.2025.04.029. Epub 2025 Jul 9.

ABSTRACT

Animal models offer a platform to advance our understanding of myasthenia gravis (MG), an autoimmune disorder characterized by impaired neuromuscular transmission. Experimental autoimmune MG models (EAMG) actively induce autoimmunity through antigen immunization, aiding in understanding the immune response to self. Passive transfer models (PTMG) involve the injection of pathogenic antibodies into animals, providing insights into antibody-mediated mechanisms and complement-driven damage to the neuromuscular junction (NMJ). The pathogenic effect of autoantibodies targeting acetylcholine receptors (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (Lrp4) results in changes to the NMJ that are mechanistically distinct. These models validate therapeutic interventions preclinically, with methodologies ranging from antibody transfer to genetic modifications. Despite the translational challenges, these models bridge preclinical research and clinical applications, enabling the development of targeted treatments for MG.

PMID:40675733 | DOI:10.1016/bs.irn.2025.04.029


Source: https://pubmed.ncbi.nlm.nih.gov/4067573 ... t9+e462414