Article
Major basic protein-1 promotes fibrosis of dystrophic muscle and attenuates the cellular immune response in muscular dystrophy.
Human molecular genetics - 1 Aug 2008
Wehling-Henricks Michelle, Sokolow Sophie, Lee Jamie J, Myung Kyu H, Villalta S Armando, Tidball James G
Abstract excerpt
The immune response to dystrophin-deficient muscle promotes the pathology of Duchenne muscular dystrophy (DMD) and the mdx mouse model of DMD. In this investigation, we find that the release of major basic protein (MBP) by eosinophils is a prominent feature of DMD and mdx dystrophy and that eosinophils lyse muscle cells in vitro by the release of MBP-1. We also show that eosinophil depletions of mdx mice by...
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