Blood-brain barrier alterations in MDX mouse, an animal model of the Duchenne muscular dystrophy.
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.
Nico
Beatrice
B
Department of Human Anatomy and Histology, University of Bari Medical School, Bari, Italy.
Roncali
Luisa
L
Mangieri
Domenica
D
Ribatti
Domenico
D
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