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Brain Res. 2011 Jun 29;1398:113-25. doi: 10.1016/j.brainres.2011.04.049. Epub 2011 May 12.

Amyotrophic lateral sclerosis: a neurovascular disease.

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Center of Excellence for Aging and Brain Repair, University of South Florida, College of Medicine, Tampa, FL 33612, USA.


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.

[Indexed for MEDLINE]

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