《生命科学》 2022, 34(2): 179-189

LGI1的结构、功能及其抗体脑炎的机制与治疗

黄万蒙¹ , 周　琳² , 沈　颖^1,*

¹浙江大学医学院生理系，杭州 310058 ²浙江大学邵逸夫医院精神卫生科，杭州 750004

摘　要：

大脑的生理功能和内稳态依赖于精细调节的突触传递和稳定的神经元兴奋性。富含亮氨酸胶质瘤失活蛋白1 (leucine rich glioma inactivated 1, LGI1) 是一种神经元分泌蛋白，与大脑发育、神经元兴奋性以及突触传递密切相关。遗传学、生物化学以及临床研究证实了LGI1 与突触传递间的关系，近期研究工作又解析了LGI1 与其受体去整合素金属蛋白酶22 (a disintegrin and metalloproteinase 22, ADAM22) 结合形成的复合体的结构，但LGI1 在疾病发生中的作用仍不清楚。该文综述了近年来LGI1 及其复合体在神经系统中的作用机制，探讨了LGI1 作为临床诊断标志物和治疗靶点在LGI1 抗体脑炎中的应用和前景，这些总结将有助于LGI1 抗体脑炎的研究和治疗。

通讯作者：沈　颖 , Email:yshen@zju.edu.cn

Structure and function of LGI1 and clinical treatment of LGI1 antibody encephalitis

HUANG Wan-Meng¹ , ZHOU Lin² , SHEN Ying^1,*

¹Department of Physiology, School of Medicine, Zhejiang University, Hangzhou 310058, China ²Department of Mental Health, Run Run Shaw Hospital, Zhejiang University, Hangzhou 750004, China

Abstract:

The physiological function and homeostasis of the brain depend on fine-tuning synaptic transmission and stable neuronal excitability. Leucine rich glioma inactivated 1 (LGI1) is a protein secreted by neurons, and is closely related to brain development, neuronal excitability, and synaptic transmission. Genetic, biochemical and clinical studies have demonstrated the relationship between LGI1 and synaptic transmission, and recent work has revealed partial structure of the binding between LGI1 and its receptor a disintegrin and metalloproteinase 22 (ADAM22). Yet, the role of LGI1 in the pathogenesis of diseases is unclear. We here review recent advance on the mechanism of LGI1 and its complex in the central nervous system, and discuss the application and prospective of LGI1 as a clinical diagnostic marker and a therapeutic target in LGI1 antibody encephalitis, which will contribute to the research and treatment of this disease.

Communication Author：SHEN Ying , Email:yshen@zju.edu.cn