Article
Immunoglobulin therapy ameliorates the phenotype and increases lifespan in the severely affected dystrophin-utrophin double knockout mice.
European journal of human genetics : EJHG - 1 Dec 2017
Nunes Bruno Ghirotto, Loures Flávio Vieira, Bueno Heloisa Maria Siqueira, Cangussu Erica Baroni, Goulart Ernesto, Coatti Giuliana Castello, Caldini Elia Garcia, Condino-Neto Antonio, Zatz Mayana
Abstract excerpt
Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder, caused by mutations in the dystrophin gene, affecting 1:3500-5000 boys worldwide. The lack of dystrophin induces degeneration of muscle cells and elicits an immune response characterized by an intensive secretion of pro-inflammatory cytokines. Immunoglobulins modulate the inflammatory response through several mechanisms and have been widely used...
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