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.
Export Options
About this article
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 |
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
Related Articles
-
Oral Hypoglycemics: A Review of Chemicals Used to Treat Type 2 Diabetes
Current Bioactive Compounds Pregnancy and Sleep Apnea
Current Respiratory Medicine Reviews Platelet-neutrophil Interactions as a Target for Prevention and Treatment of Transfusion- related Acute Lung Injury
Current Pharmaceutical Design Perspectives for Novel Mixed Diruthenium-Organic Drugs as Metallopharmaceuticals in Cancer Therapy
Anti-Cancer Agents in Medicinal Chemistry Mitochondria: A Target for Neuroprotective Interventions in Cerebral Ischemia-Reperfusion
Current Pharmaceutical Design Normobaric Hyperoxia Preconditioning Induces Changes in the Brain Lipidome
Current Neurovascular Research Neuroprotection in Hypoxic-Ischemic Brain Injury Targeting Glial Cells
Current Pharmaceutical Design Exercise-Induced Overexpression of Angiogenic Factors and Reduction of Ischemia / Reperfusion Injury in Stroke
Current Neurovascular Research Anti-VEGF Strategies – from Antibodies to Tyrosine Kinase Inhibitors: Background and Clinical Development in Human Cancer
Current Pharmaceutical Design Heart Failure in Acute Ischemic Stroke
Current Cardiology Reviews Ferrous Ion Chelating Modification and Treatment of Iron-Deficiency Anemia of Exopolysaccharide from Lachnum sp.
Current Chemical Biology The Current Status of Novel PET Radio-Pharmaceuticals in Radiotherapy Treatment Planning of Glioma
Current Pharmaceutical Biotechnology Strategies to Create a Regenerating Environment for the Injured Spinal Cord
Current Pharmaceutical Design Plasma Infusions into Porcine Cerebral White Matter Induce Early Edema, Oxidative Stress, Pro-Inflammatory Cytokine Gene Expression and DNA Fragmentation: Implications for White Matter Injury with Increased Blood- Brain-Barrier Permeability
Current Neurovascular Research n-Butyl-α-D-fructofuranoside Isolated from Ulmus davidiana Enhances Nrf2 Activity Through Activation of JNK
Current Pharmaceutical Biotechnology Anticancer Potential of Dietary Natural Products: A Comprehensive Review
Anti-Cancer Agents in Medicinal Chemistry Cardiac Imaging in Heart Failure with Comorbidities
Current Cardiology Reviews Targeting Heat Shock Protein 90 for Malaria
Mini-Reviews in Medicinal Chemistry Phytochemicals to Prevent Inflammation and Allergy
Recent Patents on Inflammation & Allergy Drug Discovery Gene Therapy in In Vivo Isolated Perfusion Models
Current Gene Therapy