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Current Neurovascular Research

Editor-in-Chief

ISSN (Print): 1567-2026
ISSN (Online): 1875-5739

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

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

Volume 2, Issue 1, 2005

Page: [47 - 54] Pages: 8

DOI: 10.2174/1567202052773481

Price: $65

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


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