Blood-brain barrier alterations in MDX mouse, an animal model of the Duchenne muscular dystrophy

Beatrice Nico, Luisa Roncali, Domenica Mangieri, Domenico Ribatti

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)47-54
Number of pages8
JournalCurrent Neurovascular Research
Volume2
Issue number1
DOIs
Publication statusPublished - Jan 2005

Keywords

  • Aquaporin-4
  • Blood brain barrier
  • Duchenne muscular dystrophy
  • Dystrophin
  • Muscular dystrophy mouse
  • Tight junctions
  • Zonula-occludens-1

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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