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
-
Concentration-Dependent Mechanisms of Adverse Drug Reactions in Epilepsy
Current Pharmaceutical Design Rhodanine as a Privileged Scaffold in Drug Discovery
Current Medicinal Chemistry Histone Deacetylase (HDAC) Inhibitors - emerging roles in neuronal memory, learning, synaptic plasticity and neural regeneration
Current Neuropharmacology Nitric Oxide and Inflammation
Current Medicinal Chemistry - Anti-Inflammatory & Anti-Allergy Agents HIV-1 TAT and IMMUNE DYSREGULATION in AIDS PATHOGENESIS: a THERAPEUTIC TARGET
Current Drug Targets Aquaporins as Targets for Drug Discovery
Current Pharmaceutical Design VEGF, a Mediator of the Effect of Experience on Hippocampal Neurogenesis
Current Alzheimer Research Nerve Injury Associated with Regional Anesthesia
Current Topics in Medicinal Chemistry Proteomic Analysis of the Kidney in Rat Biliary Cirrhosis
Current Proteomics Investigational Positive Inotropic Agents for Acute Heart Failure
Cardiovascular & Hematological Disorders-Drug Targets Hybrid PET Imaging in Neurologic Disease: PET/MRI Rather than PET/CT
Current Medical Imaging Intracranial Stents Past, Present and the Future Trend: Stents Made with Nano-particle or Nanocomposite Biomaterials
Current Medicinal Chemistry Focus on the Different Projects of Telemedicine Centered on the Elderly In France
Current Aging Science Anaemia in Diabetes: An Emerging Complication of Microvascular Disease
Current Diabetes Reviews A Short Review of Diffusion Models and their Possibilities to Enhance Imaging Biomarkers for Neurodegenerative Diseases
Current Medical Imaging A Systematic Approach to Hypertensive Urgencies and Emergencies
Current Hypertension Reviews Merging Transport Data for Choroid Plexus with Blood-Brain Barrier to Model CNS Homeostasis and Disease More Effectively
CNS & Neurological Disorders - Drug Targets Pleiotropic Effects of the Rho-kinase Inhibitor Fasudil After Subarachnoid Hemorrhage: A Review of Preclinical and Clinical Studies
Current Vascular Pharmacology Central Nervous System Agents for Ischemic Stroke: Neuroprotection Mechanisms
Central Nervous System Agents in Medicinal Chemistry Current Biological Therapies for Inflammatory Bowel Disease
Current Pharmaceutical Design