JOURNAL OF SHANGHAI JIAOTONG UNIVERSITY (MEDICAL SCIENCE) ›› 2020, Vol. 40 ›› Issue (05): 683-687.doi: 10.3969/j.issn.1674-8115.2020.05.021

• Review • Previous Articles     Next Articles

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).

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

CLC Number: