关键词: 能量代谢, 呼吸体, 线粒体疾病

Abstract:

Metabolism, including substance metabolism and energy metabolism, is one of the basic features of all living creatures.The mechanism and executor of energy metabolism were the tempting high spots of biochemical and structural researches, which have been gratifyingly accumulating through a long span of history. From identifying the respiratory chain elements to recognize the respirasome, which is believed to be the executor of cell respiration, scientists have gradually unveiled the secrets of energy metabolism. Most recently, Professor Yang from Tsinghua University has solved the 5.4 Å resolution structure of respirasome from porcine heart, assigning firstly the accurate position of most amino acid residues, presenting the detailed interaction within this huge molecular machine and providing important structural information for drug design and screening. In this review, the detoured history of respirasome recognition is introduced, and the amazing structure of respirasome is then described.