Treatment with human immunoglobulin G improves the early disease course in a mouse model of Duchenne muscular dystrophy

摘要: Duchenne muscular dystrophy (DMD) is a severe hereditary myopathy. Standard treatment by glucocorticosteroids limited because of numerous side effects. The aim this study was to test immunomodulation human immunoglobulin G (IgG) as in the experimental mouse model (mdx) DMD. 2 g/kg IgG compared albumin injected intraperitoneally mdx mice at age 3 and 7 weeks. Advanced voluntary wheel running parameters were recorded continuously. At 11 weeks, animals killed so that blood, diaphragm, lower limb muscles could be removed for quantitative PCR, histological analysis ex vivo muscle contraction tests. significantly improved performance reduced fatigability an test. Upon treatment, serum creatine kinase values diminished mRNA expression levels relevant inflammatory markers diaphragm muscles. Macrophage infiltration myopathic damage ameliorated quadriceps muscle. Collectively, demonstrates that, early disease course mice, improves diminishes inflammation Therefore, may promising approach Two monthly intraperitoneal injections 11-week phase mice. Voluntary diminished. In skeletal muscle, key such SPP1 macrophages reduced. suggests explored potential option pre-clinical long-term studies should helpful.