摘要/Abstract
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
PDF全文下载地址:
点我下载PDF
