Siffrin Volker
Experimental and Clinical Research Center, Max-Delbrück-Center for Molecular Medicine and Charité-Universitätsmedizin Berlin, Berlin, Germany.
Department of Neurology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, Berlin, Germany.
Glia. 2025 Oct;73(10):1928-1950. doi: 10.1002/glia.70044. Epub 2025 May 23.
Multiple sclerosis (MS) is the most common non-infectious inflammatory CNS disease, characterized by progressive neurodegeneration and focal demyelinated lesions. Traditionally considered an autoimmune disease, MS is driven by the immune system's attack on CNS myelin, resulting in cumulative disability. However, conventional anti-inflammatory treatments often fail to prevent progressive deterioration, particularly in the absence of overt inflammation, highlighting the need for a deeper understanding of its pathogenesis. Recent research has revealed a more complex disease mechanism involving both peripheral immune responses and intrinsic CNS factors, with glial cells playing a central role. Persistent inflammation in MS is associated with mixed active/inactive lesions dominated by microglia and astrocyte dysregulation. These glial populations exhibit maladaptive activation, contributing to failed remyelination and ongoing neurodegeneration. Transcriptomic and epigenomic alterations as well as aging further exacerbate glial dysfunction, creating a self-perpetuating cycle of inflammation and damage. Emerging evidence suggests that the interplay between peripheral immune cells and glial populations and the potential dual-use nature of molecular tools shared by the immune system and CNS disrupts homeostatic signaling, leading to a loss of tissue integrity. This review synthesizes findings on glial cell biology in MS, with a focus on microglia and astrocytes, while addressing their roles in demyelination, synapse loss, and neurodegeneration. The limitations of animal models, particularly EAE, in replicating the complexity of MS are also addressed. Finally, critical questions are outlined to guide future research into glial pathology and to identify novel therapeutic approaches targeting progressive MS.
多发性硬化症(MS)是最常见的非感染性炎症性中枢神经系统疾病,其特征为进行性神经退行性变和局灶性脱髓鞘病变。MS传统上被认为是一种自身免疫性疾病,由免疫系统对中枢神经系统髓鞘的攻击所驱动,导致累积性残疾。然而,传统的抗炎治疗往往无法预防疾病的进行性恶化,尤其是在没有明显炎症的情况下,这凸显了深入了解其发病机制的必要性。最近的研究揭示了一种更为复杂的疾病机制,涉及外周免疫反应和中枢神经系统内在因素,其中胶质细胞起着核心作用。MS中的持续性炎症与以小胶质细胞和星形胶质细胞失调为主的混合性活动/非活动病变相关。这些胶质细胞群体表现出适应不良的激活,导致髓鞘再生失败和持续的神经退行性变。转录组学和表观基因组学改变以及衰老进一步加剧了胶质细胞功能障碍,形成了炎症和损伤的自我延续循环。新出现的证据表明,外周免疫细胞与胶质细胞群体之间的相互作用以及免疫系统和中枢神经系统共享的分子工具的潜在双重用途性质破坏了稳态信号,导致组织完整性丧失。本综述综合了MS中胶质细胞生物学的研究结果,重点关注小胶质细胞和星形胶质细胞,同时探讨了它们在脱髓鞘、突触丧失和神经退行性变中的作用。还讨论了动物模型,特别是实验性自身免疫性脑脊髓炎(EAE)在复制MS复杂性方面的局限性。最后,概述了关键问题,以指导未来对胶质细胞病理学的研究,并确定针对进行性MS的新治疗方法。