上海交通大学学报（医学版）

上海交通大学学报(医学版) ›› 2020, Vol. 40 ›› Issue (5): 683-687.doi: 10.3969/j.issn.1674-8115.2020.05.021

• 综述 • 上一篇    下一篇

线粒体Lon蛋白酶及其相关疾病的研究进展

杨硕瑶1, 2，戚紫怡3，向 军4   

  1. 1. 上海交通大学仁济临床医学院，上海200127；2. 上海-渥太华联合医学院，上海 200127；3. 上海交通大学瑞金临床医学院，上海 200025；4. 上海交通大学医学院附属瑞金医院烧伤整形科，上海 200025
  • 出版日期:2020-05-28 发布日期:2020-05-28
  • 作者简介:杨硕瑶（1998—），女，本科生；电子信箱：shuoyao.yang@sjtu.edu.cn。
  • 基金资助:
    上海交通大学医学院第11期大学生创新训练计划项目（1117085）。

Research progress of mitochondrial Lon protease and its related diseases

YANG Shuo-yao1, 2, QI Zi-yi3, XIANG Jun4   

  1. 1. School of Renji Clinical Medicine, Shanghai Jiao Tong University, Shanghai 200127, China; 2. Ottawa-Shanghai Joint School of Medicine, Shanghai 200127, China; 3. School of Ruijin Clinical Medicine, Shanghai Jiao Tong University, Shanghai 200025, China; 4. Department of Burns and Plastic Surgery, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China
  • Online:2020-05-28 Published:2020-05-28
  • Supported by:
    The 11th Undergraduate Training Program for Innovation of Shanghai Jiao Tong University School of Medicine (1117085).

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摘要： Lon蛋白酶（Lon protease，LON）是一种依赖ATP的高度保守的丝氨酸蛋白酶，负责分解并清除异常蛋白质。作为细胞内环境稳定的重要执行者，LON可与蛋白酶体一同降解折叠错误及损伤蛋白质，保证体内蛋白质的高质量，尤其在线粒体功能的维护方面发挥了较大作用。同时，LON的正常功能对细胞在各种应激下的存活亦具有重要影响。近年来的研究表明，LON表达水平或活性的异常参与了神经退行性疾病、糖尿病、心肌缺血、脑缺血及肿瘤等多种疾病的发生与发展。该文就LON的结构与功能、异常表达与疾病的关系及其活性干预研究进行综述，以期为临床上相关疾病的诊治提供思路。

关键词: Lon蛋白酶, 线粒体, 蛋白质降解

Abstract:

Lon protease (LON) is a highly conserved ATP-dependent serine protease, which is responsible for the decomposition and elimination of abnormal proteins. As an important executor of intracellular environment stability, LON can degrade misfolded and damaged proteins together with proteasome to ensure the high quality of proteins in vivo, especially in the maintenance of mitochondrial function. At the same time, the physiological function of LON also has an important effect on cell survival under various stresses. Recent studies have shown that the abnormal expression or activity of LON is involved in the occurrence and development of many diseases such as neurodegenerative diseases, diabetes mellitus, myocardial ischemia, cerebral ischemia and tumors. This review focuses on the structure and the function of LON, the relationship between abnormal expression and diseases, and the research progress of LON activity intervention, in order to provide ideas for clinical diagnosis and treatment of related diseases.

Key words: Lon protease, mitochondria, protein degradation

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