Schirò Giuseppe, Di Stefano Vincenzo, Iacono Salvatore, Lupica Antonino, Brighina Filippo, Monastero Roberto, Balistreri Carmela Rita
Section of Neurology, Department of Biomedicine, Neurosciences and Advanced Diagnostics (BiND), University of Palermo, 90127 Palermo, Italy.
Cellular and Molecular Laboratory, Department of Biomedicine, Neurosciences and Advanced Diagnostics (BiND), University of Palermo, 90134 Palermo, Italy.
Brain Sci. 2022 Oct 20;12(10):1412. doi: 10.3390/brainsci12101412.
Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disease, characterized by the progressive degeneration of the upper and lower motor neurons in the cortex and spinal cord. Although the pathogenesis of ALS remains unclear, evidence concerning the role of the clonotypic immune system is growing. Adaptive immunity cells often appear changed in number, or in terms of their activation profiles, both peripherally and centrally; however, their role in ALS appears conflictive. Data from human and animal model studies, which are currently reported in the literature, show that each subset of lymphocytes and their mediators may mediate a protective or toxic mechanism in ALS, affecting both its progression and risk of death. In the present review, an attempt is made to shed light on the actual role of cellular clonotypic immunity in ALS by integrating recent clinical studies and experimental observations.
肌萎缩侧索硬化症（ALS）是一种致命的神经肌肉疾病，其特征是大脑皮层和脊髓中的上下运动神经元进行性退化。尽管ALS的发病机制尚不清楚，但有关克隆型免疫系统作用的证据越来越多。适应性免疫细胞在外周和中枢的数量或激活状态常常出现变化；然而，它们在ALS中的作用似乎存在矛盾。目前文献报道的来自人类和动物模型研究的数据表明，淋巴细胞的每个亚群及其介质可能在ALS中介导一种保护或毒性机制，影响其进展和死亡风险。在本综述中，我们试图通过整合最近的临床研究和实验观察结果，来阐明细胞克隆型免疫在ALS中的实际作用。
