Center of Excellence for Aging and Brain Repair, University of South Florida, College of Medicine, Tampa, FL 33612, USA.
Brain Res. 2011 Jun 29;1398:113-25. doi: 10.1016/j.brainres.2011.04.049. Epub 2011 May 12.
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.
肌萎缩侧索硬化症（ALS）是一种严重的神经退行性疾病，其发病机制复杂。大量证据表明，在患者和动物模型中，所有神经血管单元成分（包括血脑和血脊髓屏障[BBB/BSCB]）都受到损害，这导致将 ALS 归类为神经血管疾病。本综述提供了对正常和受损的 BBB/BSCB 的最新分析，重点介绍了 ALS 改变的屏障。在这里，我们描述了细胞成分、紧密连接、转运系统、细胞相互作用、细胞因子、基质金属蛋白酶和自由基在 BBB/BSCB 破坏中的作用，以及来自实验和临床 ALS 研究的最新证据。BBB/BSCB 是 ALS 中一个很有前途的研究领域，本综述将揭示 ALS 中小血管病理学的一些方面，并希望为新的治疗策略的发展提供思路。
