线粒体铁代谢与人类疾病的基础研究  

Mitochondrial iron metabolism and human disease

作　　者：李宽钰[1] 

机构地区：[1]南京大学医学院基础部铁代谢实验室/江苏省医学分子技术重点实验室,南京210093

出　　处：《医学研究生学报》2016年第1期24-28,共5页Journal of Medical Postgraduates

基　　金：国家自然科学基金(31571218;31371060);国家重点基础研究发展计划973项目(2015CB856300)

摘　　要：铁是生命所必需的元素,而游离的铁又具有毒性。细胞内过多的铁可诱导产生大量的活性氧给细胞带来致命性损伤,所以铁在细胞内的代谢受到严格的调控。线粒体是细胞内最大的铁代谢细胞器,主要负责血红素和铁硫簇的合成。血红素和铁硫簇是很重要的2种辅基,参与DNA的合成与修复,蛋白的合成与折叠,三羧酸循环,线粒体电子传递链的正常进行等重要代谢过程。线粒体内铁代谢的紊乱会严重地影响到整个细胞的铁代谢以及能量代谢,从而影响线粒体的功能,导致各种疾病的发生。文章就铁代谢和线粒体功能相关研究的工作特别是对最近1年以内发表的文章相关内容作一总结和展望。Iron is essential for virtually all organisms. It is not only essential but also toxic at higher levels, so that cells have developed sophisticated systems for ensuring a tightly regulated iron homeostasis. Iron overloading may bring about large amount of re- active oxygen species to damage the cells. On the other hand, iron depletion will restrict many chemical redox reactions. Mitochondrion is the largest organelle for cellular iron metabolism, mainly for berne and iron sulfur （Fe-S） cluster biogenesis. Both heme and Fe-S are important cofactors involved in central cellular processes such as DNA synthesis and repair, ribosome biogenesis, tricarboxylic acid cycle, and respiration （electron transfer chain）. The imbalance of mitoehondrial iron metabolism involves the cellular iron homeosta- sis, energy metabolism, and ultimately mitochondrial failure and disease occurrence. Our laboratory focuses on basic research, expands to pre-clinical or clinical study, linking iron metabolism and mitochondrial function with diseases. Here is a summary of our recent work, in particular the work published last year.

关 键 词：铁硫簇 线粒体的功能 铁代谢疾病 

分 类 号：R364[医药卫生—病理学]