Elimination of intramuscular immunoglobulin accumulation alleviates Duchenne Muscular Dystrophy
Chang, X.; Chen, Y.; Zhang, L.; Guo, W.; Huang, M.; Liang, Z.; Zhu, E.; Li, G.; Qi, T.; Chang, C.; Guo, X.; Sun, W.; Li, J.; Liu, J.; Qiu, H.; Zhu, L.; Liu, Y.; Chen, J.; Ren, S.; Bai, R.; Wang, J.; Gao, Y.; Song, Y.; Li, F.; Dai, Y.; Wu, Z.; Hu, J.; Ji, W.
Show abstract
Duchenne muscular dystrophy (DMD) is a devastating neuromuscular disorder due to loss of dystrophin, a cytoskeletal protein critical for muscle integrity and functionality. Despite recent therapeutic advances, there remains a significant unmet need for more effective and accessible therapeutics. Here, we discovered an early accumulation of immunoglobulin G (IgG) in the sarcolemma, which exacerbates tissue inflammation and disease progression. The IgG accumulation primarily resulted from ectopic localization of Fc{gamma}R1, a high-affinity IgG Fc receptor, on dystrophin-deficient myofibers. In two independent murine models, eliminating IgG accumulation via B cell depletion provided sustained benefit to alleviate DMD progression. Our findings uncover a novel disease accelerator in DMD and demonstrate the potential to target this mechanism as therapeutics for broader population of patients with DMD.
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