title: Blood-brain barrier alterations in MDX mouse, an animal model of the Duchenne muscular dystrophy.
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 dysfunctions of mdx mice and DMD patients could be associated with changes in brain osmotic equilibrium.
authors.affiliation: Department of Human Anatomy and Histology, University of Bari Medical School, Bari, Italy.
authors.forename: Beatrice
authors.initials: B
authors.lastname: Nico
authors.forename: Luisa
authors.initials: L
authors.lastname: Roncali
authors.forename: Domenica
authors.initials: D
authors.lastname: Mangieri
authors.forename: Domenico
authors.initials: D
authors.lastname: Ribatti
mesh_terms: D000818:Animals; D001812:Blood-Brain Barrier; D004195:Disease Models, Animal; D051379:Mice; D018101:Mice, Inbred mdx; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism* / Q000503:physiopathology; D012376:Rodent Diseases / Q000378:metabolism* / Q000503:physiopathology