视神经蛋白的功能及其在神经退行性疾病中的作用

郑若玲 谭洁

yl6809永利皇宫广西脑与认知神经科学重点实验室

摘要：视神经蛋白（OPTN）是一种在人体多器官组织中表达的高丰度蛋白质，其通过与多种蛋白质作用参与自噬、囊泡运输、炎症反应等胞内过程。而OPTN的突变是多种神经退行性疾病的致病因素。本文总结了OPTN的蛋白结构、表达情况、生物学功能及OPTN在青光眼、肌萎缩侧索硬化症（ALS）及帕金森病（PD）等神经退行性疾病发生发展中的作用，为治疗神经退行性疾病提供新的参考。

关键词：视神经蛋白;自噬;神经退行性疾病;

The function of optineurin and its research progress in neurodegenerative diseases①

ZHENG Ruoling,TAN Jie②.(Guangxi Key Laboratory of Brain and Cognitive Neuroscience,Guilin Medical University,Guilin 541199,China)

Abstract Optineurin (OPTN) is widely distributed in various tissues and organs in human. It is involved in multiple intracellular processes such as autophagy, vesicle transport, and inflammatory response by binding to a variety of proteins. The mutations of OPTN is the pathogenic factors of various neurodegenerative diseases. This review summarizes the structure, expression pattern, biological function of OPTN and the research progress of its role in the formation and development of neurodegenerative diseases such as Glaucoma, Amyotrophic lateral sclerosis (ALS) and Parkinson′s disease (PD), aiming to offer new ideas for the treatment for neurodegenerative diseases.

Keywords: optineurin; autophagy; neurodegenerative disease