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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 clusters are a class of ancient and multi-functional protein cofactors involved in the process of electron transfer, enzymatic reactions and changes in the perception of the internal environment to regulate gene expression. Although the composition and structure of iron-sulfur clusters are relatively simple, the assembly of iron-sulfur clusters requires a variety of assembly proteins involved in the orderly catalytic reaction. It was only in recent years that people gradually elucidated how iron-sulfur clusters are assembled and incorporated into immature iron-sulfur proteins in living organisms. If the mitochondrial iron-sulfur cluster assembly and transport process obstacles, will seriously affect the intracellular iron homeostasis and iron-sulfur protein function, we can see that the assembly of iron and sulfur mitochondria make mitochondria become an essential class of cells Organelles. This review focuses on the recent progress in the assembly of iron and sulfur clusters in eukaryotes and describes the important role of mitochondrial iron-sulfur clusters in the human body and the disorders caused by their assembly disruption.