真核细胞中铁硫簇的组装机制及相关铁硫蛋白疾病-中国细胞生物学学报.PDF

网络出版时间：2015-09-18 17:02:38 网络出版地址：/kcms/detail/31.2035.Q1702.006.html 中国细胞生物学学报 Chinese Journal of Cell Biology 2015, 37(9): DOI: 10.11844/cj cb.2015.09.012 1 真核细胞中铁硫簇的组装机制及相关铁硫蛋白疾病 * * 杜 李艳纯 任雪营 谭国强 吕建新 (温州医科大学检验医学院、生命科学学院, 浙江省医学遗传学重点实验室, 温州325035) 摘要 铁硫簇是一类古老而功能众多的蛋白质辅基, 在细胞中参与电子传递过程、酶促反 应及感知内环境的变化而调节基因的表达等。虽然铁硫簇的组成元素和结构都较为简单, 但是铁 硫簇的组装是需多种组装蛋白参与、有序进行的催化反应。直至近几年, 人们才逐渐阐明了在生 命体中铁硫簇是如何组装并结合到未成熟的铁硫蛋白中的。如果线粒体中铁硫簇组装及转运过程 发生障碍, 将严重影响细胞内铁的稳态及铁硫蛋白的功能, 由此可见, 线粒体中铁硫簇的组装功能 使得线粒体成为细胞中必不可少的一类细胞器。本文将重点概述近十年来真核生物中铁硫簇组装 机制研究进展并阐述线粒体铁硫簇组装在人体中的重要作用及其组装障碍所引起的疾病。 关键词 铁硫簇; 铁硫蛋白; 铁调节; 铁硫蛋白病 Mechanisms of Iron-sulfur Clusters Assemble in Eukaryotes and Related Diseases Du Jing, Li Yanchun, Ren Xueying, Tan Guoqiang*, Lü Jianxin* (School of Laboratory Medicine and Life Sciences, Zhejiang Provincial Key Laboratory of Medical Genetics, Wenzhou Medical University, Wenzhou 325035, China) Abstract Iron-sulfur (Fe-S) cluster is an ancient protein cofactor which has been known with many functions, and it involves in many physiological processes, such as catalysis, electron transport and regulation of gene expression. Despite the simplicity of its structure and composition, Iron-sulfur cluster (ISC) has an complicated assembling process which is a well-organized catalytic reaction. Over the past years the mechanisms of the process of iron-sulfur clusters’ assembly and incorporation into apoproteins have been gradually clarified. Defects in the mitochondrial Iron-