Journal
BRAIN RESEARCH
Volume 1398, Issue -, Pages 113-125Publisher
ELSEVIER
DOI: 10.1016/j.brainres.2011.04.049
Keywords
Amyotrophic lateral sclerosis; Blood-brain barrier (BBB); Blood-spinal cord barrier (BSCB); Impairment; Microvascular pathology
Categories
Funding
- Muscular Dystrophy Association [92452]
- James and Esther King Biomedical Research Program [1KG01-33966]
- USF Department of Neurosurgery and Brain Repair
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Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative disease with a complicated pathogenesis. Compelling evidence indicates impairment of all neurovascular unit components including the blood-brain and blood-spinal cord barriers (BBB/BSCB) in both patients and animal models, leading to classification of ALS as a neurovascular disease. The present review provides an updated analysis of the normal and impaired BBB/BSCB, focusing on the ALS-altered barrier. Here we describe the roles of cellular components, tight junctions, transport systems, cell interactions, cytokines, matrix metalloproteinases, and free radicals in the BBB/BSCB disruption, along with recent evidence from experimental and clinical ALS studies. The BBB/BSCB is a promising research area in ALS and this review will reveal some aspects of microvascular pathology in ALS and hopefully provide ideas for the development of new therapeutic strategies. (C) 2011 Elsevier B.V. All rights reserved.
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