作者
Dervla O’Malley
发表日期
2016
简介
Duchenne Muscular Dystrophy (DMD) is an X chromosome-linked disease characterized by 22 progressive physical disability, immobility and premature death in affected boys. Underlying 23 the devastating symptoms of DMD is the loss of dystrophin, a structural protein which 24 connects the extracellular matrix to the cell cytoskeleton and provides protection against 25 contraction-induced damage in muscle cells, leading to chronic peripheral inflammation. 26 However, dystrophin is also expressed in neurons within specific brain regions, including the 27 hippocampus, a structure associated with learning and memory formation. Linked to this, a 28 subset of boys with DMD exhibit non-progressing cognitive dysfunction, with deficits in 29 verbal, short-term and working memory. Furthermore, in the genetically comparable 30 dystrophin-deficient mdx mouse model of DMD, some, but not all, types of learning and 31 …