Abstract
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
Current Neurovascular Research
Title: Blood-Brain Barrier Alterations in MDX Mouse, An Animal Model of the Duchenne Muscular Dystrophy
Volume: 2 Issue: 1
Author(s): Beatrice Nico, Luisa Roncali, Domenica Mangieri and Domenico Ribatti
Affiliation:
Keywords: aquaporin-4, blood brain barrier, dystrophin, duchenne muscular dystrophy, muscular dystrophy mouse, tight junctions, zonula-occludens-1
Abstract: 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.
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Cite this article as:
Nico Beatrice, Roncali Luisa, Mangieri Domenica and Ribatti Domenico, Blood-Brain Barrier Alterations in MDX Mouse, An Animal Model of the Duchenne Muscular Dystrophy, Current Neurovascular Research 2005; 2 (1) . https://dx.doi.org/10.2174/1567202052773481
DOI https://dx.doi.org/10.2174/1567202052773481 |
Print ISSN 1567-2026 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5739 |
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