视神经脊髓炎的发病机制

The Pathogensis of Neuromyelitis Optica

视神经脊髓炎免疫球蛋白G(NMO-Ig G)作用于水通道蛋白-4(AQP4),使AQP4含量减少并产生抗AQP4抗体。透过受损的血脑屏障,使NMO-Ig G进入中枢神经系统内,抗AQP4抗体损害了AQP4和Na+-依赖兴奋性氨基酸转运体复合物,引起谷氨酸兴奋性毒性和血脑屏障破坏。抗AQP4抗体通过细胞免疫毒性和补体依赖性细胞毒性、化学因素(过敏毒素如C3a、C5a)引起星形细胞损害。所有机制综合性损害血脑屏障,增加抗体,各种细胞(淋巴细胞和粒细胞)侵入中枢神经系统,造成视神经脊髓炎。

After the break down of aquaporin-4（AQP4） periperal tolerance leading to neuromyelitis optica immunoglobulin G （NMO-IgG）/anti-AQP4 antibody production, a beach in the blood-brain barriar （BBB）, allows NMO-IgG to reach the CNS, bind AQP4 and induce pathogenic fuction. Anti-AQP4 antibodies induce the internalization of AQP4 and EAAT2 astrocyte proteins, and result in excitetoxicity, and increase BBB permeability. Anti-AQP4 antibodies can result in astrocyte injury by antibody dependent cellular cytotocity. Chemotactic factors such as anaphylatoxins C3a and C5a are released and induce the recruitment and activation of inlfammatory cells. All these mechanisms futher contribute to BBB disruption, enhancing antibody and cell entry to the CNS, and propagating NMO lesion formation.