真核细胞中铁硫簇的组装机制及相关铁硫蛋白疾病

Mechanisms of Iron-sulfur Clusters Assemble in Eukaryotes and Related Diseases

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。该文重点概述了近十年来真核生物中铁硫簇组装机制的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。

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-sulfur cluster assembly and export systems have a powerful impact on cellular Iron-sulfur protein＇s function and intracellular iron distribution. These provided strong evidences for the indispensable role of mitochondrial in life. In this review, we have summarized our current knowledge about the ISC assembly machinery briefly, and presented an overview of various （Fe-S） protein assembly diseases.