补体信号通路在视神经脊髓炎发病机制中的作用研究现状

The role of complement signaling pathway in the pathogenesis of neuromyelitis optica

视神经脊髓炎(NMO)是一种选择性累及视神经和脊髓的中枢神经系统脱髓鞘疾病,其发病机制为水通道蛋白4(AQP4)抗体与星形胶质细胞足突上AQP4结合后,引发补体依赖的细胞毒性、抗体依赖的细胞介导的细胞毒性及其下游的一系列炎症反应,从而导致少突胶质细胞和神经元的损害。被激活的补体以血管为中心而沉积,成为NMO的一个显著的病理特点。近年多个研究中心发现补体在NMO发病过程中起着至关重要的作用,补体抑制剂可以有效缓解病情而达到治疗的目的。全面、系统了解补体信号通路在NMO发病机制中的作用,为更加深入的了解NMO及其治疗提供参考。

Neuromyelitis optica (NMO) is a kind of demyelinating disorder that preferentially affects the optic nerves and spinal cord and results in permanent vision loss. NMO pathogenesis is thought to involve binding of anti-aquaporin-4 (AQP4) auto-antibodies to astrocytes, which causes complement-dependent cytotoxicity (CDC) and downstream inflammation leading to oligodendrocyte and neuronal injury. Vasculocentric deposition of activated complement is a prominent feature of NMO pathology. In recent years, a number of groups have found complements play an important role in the pathogenesis of NMO, and basic researches in NMO therapy due to its specificity and uniformity. Its inhibition would protect against proteins in the classical complement pathway so that cure the disease. This review will expound the the role of complement signaling pathway in the pathogenesis of NMO, and provide reference for a more in-depth understanding and clinical treatments of NMO.