Guan Shuzhen, Zhu Huimin, Zhang Mengting, Shi Fu-Dong, Yan Bo
Department of Neurology, Tianjin Neurological Institute, Tianjin Medical University General Hospital, Tianjin, China.
Department of Neurology, China National Clinical Research Center for Neurological Disease, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Cell Death Differ. 2025 Sep 9. doi: 10.1038/s41418-025-01576-7.
Multiple sclerosis (MS) is a chronic autoimmune disorder of the central nervous system (CNS) characterized by inflammatory demyelination and progressive neurodegeneration. Although current disease-modifying therapies modulate peripheral autoimmune responses, they are insufficient to fully prevent tissue specific neuroinflammation and long-term neuronal and oligodendrocyte loss. Growing evidence implicates various regulated cell death (RCD) pathways, including apoptosis, necroptosis, pyroptosis, and ferroptosis, not only as downstream consequences of chronic inflammation, but also as active drivers of demyelination, axonal injury, and glial dysfunction in MS. These RCD modalities contribute to MS pathology by disrupting cellular homeostasis and sustaining immune activation through the continuous release of damage-associated molecular patterns (DAMPs), thereby establishing a self-amplifying loop between cell death and inflammation. Furthermore, distinct RCD forms can co-occur within lesions, contributing to the complex cellular landscape of MS. This review summarizes current understanding of RCD mechanisms in MS, focusing on their contributions to neuroinflammation and neurodegeneration across different disease stages. We also discuss recent therapeutic advances targeting RCD, including approved drugs whose efficacy may be partially attributed to modulation of cell death, and emerging small-molecule inhibitors targeting key cell death components such as receptor-interacting protein kinase 1 (RIPK1) and NOD-, leucine-rich repeat-, and pyrin domain-containing protein 3 (NLRP3). Targeting RCD in conjunction with inflammation may represent a more pragmatic approach for mitigating MS progression and neurodegeneration.
多发性硬化症(MS)是一种中枢神经系统(CNS)的慢性自身免疫性疾病,其特征为炎症性脱髓鞘和进行性神经退行性变。尽管目前的疾病修正疗法可调节外周自身免疫反应,但它们不足以完全预防组织特异性神经炎症以及长期的神经元和少突胶质细胞损失。越来越多的证据表明,包括凋亡、坏死性凋亡、焦亡和铁死亡在内的各种程序性细胞死亡(RCD)途径,不仅是慢性炎症的下游后果,也是MS中脱髓鞘、轴突损伤和神经胶质功能障碍的主动驱动因素。这些RCD模式通过破坏细胞内稳态并通过持续释放损伤相关分子模式(DAMPs)维持免疫激活,从而导致MS病理改变,进而在细胞死亡和炎症之间建立一个自我放大的循环。此外,不同的RCD形式可在病灶内共同出现,导致MS复杂的细胞格局。本综述总结了目前对MS中RCD机制的理解,重点关注它们在不同疾病阶段对神经炎症和神经退行性变的作用。我们还讨论了针对RCD的最新治疗进展,包括其疗效可能部分归因于细胞死亡调节的已批准药物,以及针对关键细胞死亡成分如受体相互作用蛋白激酶1(RIPK1)和含核苷酸结合寡聚化结构域样受体蛋白3(NLRP3)的新型小分子抑制剂。联合炎症靶向RCD可能是减缓MS进展和神经退行性变的更实用方法。