Immunogenetics of experimental autoimmune myasthenia gravis

Myasthenia gravis (MG) is an autoimmune neuromuscular disease manifested by muscle weakness and fatiguability. The primary pathology in MG is antibody and complement-mediated destruction of muscle acetylcholine receptor (AChR). Like other autoimmune diseases, MG is associated with certain HLA antigens, particularly HLA-B8 and DR3 in Caucasians. Also, certain GM antigens and complotypes are associated with MG. Therefore, it is crucial to study the immunogenetic aspect of MG in animal models to evaluate disease etiopathogenesis and eventual strategy for specific therapy. In the introduction of this review article, I focus on the association of HLA and GM antigens in MG and emphasize the mouse model of experimental autoimmune myasthenia gravis (EAMG) as an ideal model to study the immunogenetic aspect of MG. The following sections deal with the role of (1) major histocompatibility complex (MHC), (2) immune response gene, (3) the IA molecule, (4) the Igl locus, (5) the complement genes, and (6) non-MHC genes on EAMG pathogenesis. The review concludes with future immunogenetic analysis and eventual strategy for specific therapy from an immunogeneticist's point of view.