Department of Genetics and Genome Sciences, Case Western Reserve University, Cleveland, Ohio, USA.
Human Genome and Stem Cell Research Center, Biosciences Institute, University of São Paulo, São Paulo, Brazil.
Adv Exp Med Biol. 2019;1147:137-146. doi: 10.1007/978-3-030-16908-4_6.
In amyotrophic lateral sclerosis (ALS), motor neurons die selectively. Therefore, initial symptoms that include fasciculation, spasticity, muscle atrophy, and weakness emerge following axons retraction and consequent muscles' denervation. Patients lose the ability to talk and swallow and rely on parenteral nutrition and assisted ventilation to survive. The degeneration caused by ALS is progressive and irreversible. In addition to the autonomous mechanism of neuronal cell death, non-autonomous mechanisms have been proved to be toxic for motor neurons, such as the activation of astrocytes and microglia. Among the cells being studied to unveil these toxic mechanisms are pericytes, cells that help keep the integrity of the blood-brain barrier and blood-spinal cord barrier. In this chapter, we aim to discuss the role of pericytes in ALS.
在肌萎缩侧索硬化症（ALS）中，运动神经元选择性死亡。因此，包括肌束颤动、痉挛、肌肉萎缩和无力在内的初始症状出现在轴突回缩和随后的肌肉失神经支配之后。患者丧失说话和吞咽能力，依赖肠外营养和辅助通气来维持生命。ALS 引起的退行性变是进行性和不可逆转的。除了神经元细胞死亡的自主机制外，非自主机制已被证明对运动神经元有毒，如星形胶质细胞和小胶质细胞的激活。在研究揭示这些毒性机制的细胞中，周细胞有助于保持血脑屏障和血脊髓屏障的完整性。在本章中，我们旨在讨论周细胞在 ALS 中的作用。
