| Objective:To establish an experimental autoimmune myasthenia gravis (EAMG) model with the similar myasthenia gravis (MG) symptoms in human and to explore the pathogenicity of muscle-specific kinase (MuSK) antibody (MuSKAb) in MGMethods:Lewis rats were divided into experimental group and control group. The experimental group was injected with MuSK monoclonal antibodies. The control group was injected with phosphate buffer saline (PBS) at the same time. All rats were observed every day until96hours. At the observed time, the clinical symptoms and the changes of weight were recorded. After96hours, all the rats were executed and the intercostals, gastrocnemius and pretibial muscles were got. Anatomical muscles were frozen at-80℃. Then immunofluorescence was done to observe whether MuSKAb bound MuSK and the ultrastructure of neuromuscular junctions (NMJ) were observed with transmission electronic microscope.Results:Experimental group was compared with the control group after96hours. There was no obvious clinical symptom, myodynamia decline, but there was difference in ultrastructure. The yellow-green fluorescence and the red fluorescence on the cell membrane of experimental group were observed. But no fluorescence was observed in control group. The yellow-green fluorescence showed that MuSKAb bound MuSK on the cell membrane and the red fluorescence showed that immunocomplex activated C3complement. The ultrastructure of NMJ showed particular pathological changes, such as the less synaptic folds and the larger synaptic cleft, similar to clinical MG.Conclusion:Injection of MuSK monoclonal antibodies fails to induce clinical symptoms in rats. However, MuSKAb can bind to MuSK and activate complement C3on the cell membrane of muscles, and futhermore there exist morpholygical changes of ultrastructure in NMJ. The pathogenicity of MuSKAb in MG needs to be further studied in the future. |
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