Abstract
Congenital Muscular Dystrophy type 1D (CMD1D) is characterized by an abnormal glycosylation of α-DG (α-dystroglycan) and is associated to the central nervous system (CNS) abnormalities such as cognitive impairment. The purpose of the research was to evaluate the blood-brain barrier permeability (BBB) permeability and matrix metalloproteinases (MMP) -2 and -9 in adult Largemyd-/- mice in order to understand the physiopathology of brain involvement during the CMD1D process. To this aim, we used adult homozygous Largemyd-/- (mutation in Large), heterozygous Largemyd+/- as well as wild-type (C57BL/6) mice. The animals were submitted to the evaluation of BBB permeability and MMP-2 and MMP-9 in striatum, hippocampus and cerebral cortex. There was an increase in BBB permeability in the hippocampus and striatum associated with an increase in the protein levels of MMP-2 in the cerebral cortex and striatum and MMP-9 in the hippocampus in adult Largemyd-/- mice. Our results suggest that the pathophysiologic processes can be associated to the action of MMPs and BBB disruption and that the BBB breakdown is relevant to the perpetuation of brain inflammation and can be related to brain dysfunction observed in CMD1D patients.
Keywords: Congenital Muscular Dystrophy type 1D, Large, brain, blood-brain barrier permeability, matrix metalloproteinases.
Current Neurovascular Research
Title:Congenital Muscular Dystrophy 1D Causes Matrix Metalloproteinase Activation And Blood-Brain Barrier Impairment
Volume: 14 Issue: 1
Author(s): Aryadnne L. Schactae, Daphne Palmas, Monique Michels, Jaqueline S. Generoso, Tatiana Barichello, Felipe Dal-Pizzol, Mariz Vainzof and Clarissa M. Comim
Affiliation:
Keywords: Congenital Muscular Dystrophy type 1D, Large, brain, blood-brain barrier permeability, matrix metalloproteinases.
Abstract: Congenital Muscular Dystrophy type 1D (CMD1D) is characterized by an abnormal glycosylation of α-DG (α-dystroglycan) and is associated to the central nervous system (CNS) abnormalities such as cognitive impairment. The purpose of the research was to evaluate the blood-brain barrier permeability (BBB) permeability and matrix metalloproteinases (MMP) -2 and -9 in adult Largemyd-/- mice in order to understand the physiopathology of brain involvement during the CMD1D process. To this aim, we used adult homozygous Largemyd-/- (mutation in Large), heterozygous Largemyd+/- as well as wild-type (C57BL/6) mice. The animals were submitted to the evaluation of BBB permeability and MMP-2 and MMP-9 in striatum, hippocampus and cerebral cortex. There was an increase in BBB permeability in the hippocampus and striatum associated with an increase in the protein levels of MMP-2 in the cerebral cortex and striatum and MMP-9 in the hippocampus in adult Largemyd-/- mice. Our results suggest that the pathophysiologic processes can be associated to the action of MMPs and BBB disruption and that the BBB breakdown is relevant to the perpetuation of brain inflammation and can be related to brain dysfunction observed in CMD1D patients.
Export Options
About this article
Cite this article as:
Schactae L. Aryadnne, Palmas Daphne, Michels Monique, Generoso S. Jaqueline, Barichello Tatiana, Dal-Pizzol Felipe, Vainzof Mariz and Comim M. Clarissa, Congenital Muscular Dystrophy 1D Causes Matrix Metalloproteinase Activation And Blood-Brain Barrier Impairment, Current Neurovascular Research 2017; 14 (1) . https://dx.doi.org/10.2174/1567202613666161201204549
DOI https://dx.doi.org/10.2174/1567202613666161201204549 |
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
-
Treatment of Epileptic Encephalopathies
Current Pharmaceutical Design Genetic Studies in Relation to Kuru: An Overview
Current Molecular Medicine Pregabalin Treatment does not Affect Amyloid Pathology in 5XFAD Mice
Current Alzheimer Research Glycogen Synthase Kinase-3: A Potential Target for Drug Discovery in the Treatment of Neurodegenerative Disorders
Current Enzyme Inhibition Size-tuneable Nanometric MRI Contrast Agents for the Imaging of Molecular Weight Dependent Transport Processes
Pharmaceutical Nanotechnology The Response of the Aged Brain to Stroke: Too Much, Too Soon?
Current Neurovascular Research Drug Abuse, Brain Calcification and Glutamate-Induced Neurodegeneration
Current Drug Abuse Reviews Benefits of Caloric Restriction on Brain Aging and Related Pathological States: Understanding Mechanisms to Devise Novel Therapies
Current Medicinal Chemistry Glucose Transporters Regulation on Ischemic Brain: Possible Role as Therapeutic Target
Central Nervous System Agents in Medicinal Chemistry ROCK in CNS: Different Roles of Isoforms and Therapeutic Target for Neurodegenerative Disorders
Current Drug Targets The Seek of Neuroprotection: Introducing Cannabinoids
Recent Patents on CNS Drug Discovery (Discontinued) Neurological Substrate of Central Auditory Processing Deficits in Children
Current Pediatric Reviews Proteasome Modulation in Brain: A New Target for Anti-Aging Drugs?
Central Nervous System Agents in Medicinal Chemistry Carvedilol: Just Another Beta-Blocker or a Powerful Cardioprotector?
Cardiovascular & Hematological Disorders-Drug Targets Cerebrovascular Amyloidosis and Dementia
Current Medicinal Chemistry - Immunology, Endocrine & Metabolic Agents Glycine Site Modulators and Glycine Transporter-1 Inhibitors as Novel Therapeutic Targets for the Treatment of Schizophrenia
Current Neuropharmacology MicroRNAs: Key Players in Microglia and Astrocyte Mediated Inflammation in CNS Pathologies
Current Medicinal Chemistry Diagnostic Approach to Mitochondrial Disorders: the Need for a Reliable Biomarker
Current Molecular Medicine Role of Connexins and Pannexins in Ischemic Stroke
Current Medicinal Chemistry COMMENTARY: Transcranial Magnetic Stimulation in Multiple Sclerosis: A Method to Improve Movement
CNS & Neurological Disorders - Drug Targets