Blood-Brain Barrier Alterations in MDX Mouse, An Animal Model of the Duchenne Muscular Dystrophy

Author(s): Beatrice Nico, Luisa Roncali, Domenica Mangieri, Domenico Ribatti

Journal Name: Current Neurovascular Research

Volume 2 , Issue 1 , 2005

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This article reviews recent studies on the alterations occurring in the brain vessel wall of the mdx mouse, an animal model with genetic defects in a region homologous with the human Duchenne muscular dystrophy (DMD) gene. These alterations affect both endothelial and astroglial cells and are associated with opened tight junctions, swollen perivascular astrocyte processes and a reduction in the expression of tight junctions associated proteins, ie. zonula occludens and of a specific water channel i.e. aquaporin-4, suggesting that some neurological dyspfunctions of mdx mice and DMD patients could be associated with changes in brain osmotic equilibrium.

Keywords: aquaporin-4, blood brain barrier, dystrophin, duchenne muscular dystrophy, muscular dystrophy mouse, tight junctions, zonula-occludens-1

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Article Details

Year: 2005
Page: [47 - 54]
Pages: 8
DOI: 10.2174/1567202052773481

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