Myasthenia Gravis (MG) Models
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Myasthenia Gravis (MG) Models
Myasthenia Gravis (MG) is a chronic autoimmune neuromuscular disorder characterized by weakness and fatigability of skeletal muscles due to autoantibodies targeting components of the neuromuscular junction, most commonly the acetylcholine receptor (AChR).
In preclinical studies, the most commonly used MG model is AChRα97-116 Experimental Autoimmune Myasthenia Gravis (EAMG), which is induced in rodents either by active immunization with purified AChR or by passive transfer of anti-AChR antibodies. These models reproduce key disease characteristics, including antibody-mediated receptor degradation, complement activation, and muscle weakness. MG models are essential for dissecting disease mechanisms, understanding immune tolerance breakdown, and evaluating novel immunomodulatory and symptomatic therapies. Besides, we also developed a MG patient derived PBLs NCG model to meet your MG research requirement.
For more information:
AChRα97-116 induced EAMG Models
Fig. 1: Development and Validation of AChRα97–116-Induced EAMG Model.
(A) Experimental autoimmune myasthenia gravis (EAMG) was induced by immunizing animals with the AChRα97–116 peptide at weeks 0, 5, 8, and 11, with forelimb grip strength monitored over time.
(B) A significant reduction in forelimb grip strength was observed in the model group, indicating progressive muscle weakness.
(C) Serum levels of total mouse IgG and anti-AChR antibodies increased from week 5 to 12 post-induction, correlating with elevated serum TNF-α levels.
(D) An increase in spleen weight index and a decrease in thymus and gastrocnemius muscle weight indices were recorded in the disease group.
(E) Flow cytometry analysis revealed a significant increase in the percentages of T follicular helper (Tfh) cells, IFN-γ–producing CD4⁺ T cells, and IL-17A–producing CD4⁺ T cells.
(F) Histopathological evaluation (HE score) was significantly elevated in the model group. H&E staining showed muscle fiber atrophy, inflammatory cell infiltration, and areas of fibrosis and necrosis.
Patient derived PBL-NCG MG Model
The Patient-Derived PBL-NCG MG Model is a humanized mouse model for studying Myasthenia Gravis (MG), created by engrafting peripheral blood lymphocytes (PBLs) from MG patients into immunodeficient NCG mice (Strain No. T001475). Following engraftment, the mice develop functional human T and B cell populations capable of producing anti-acetylcholine receptor (AChR) autoantibodies, closely replicating the human MG immune profile. After 4-7 weeks, these mice exhibit hallmark features of MG, including muscle weakness, reduced grip strength, and IgG/complement deposition at the neuromuscular junction. This model provides a clinically relevant platform for investigating MG pathogenesis and evaluating human-specific immunotherapies.
Fig. 2: Development and Validation of the Patient-Derived PBL-NCG MG Model.
At 4–7 weeks post-injection of PBLs from Myasthenia Gravis (MG) patients into NCG mice, NCG mice exhibit muscle weakness, evidenced by reduced forelimb limb grip strength. Histological analysis of the tibialis anterior muscle demonstrated muscle fiber degeneration accompanied by substantial inflammatory cell infiltration. These pathological features correlate with significantly elevated levels of human AChR autoantibodies and total IgG in the serum.
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