首页|Mammalian mitochondrial iron-sulfur cluster biogenesis and transfer and related human diseases
Mammalian mitochondrial iron-sulfur cluster biogenesis and transfer and related human diseases
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As a cofactor,iron-sulfur (Fe-S) cluster binds to proteins or enzymes that play important roles in vari-ous important biological processes,including DNA synthesis and repair,mitochondrial function,gene transcription and translation.In mammals,the core components involved in Fe-S cluster biosynthesis are considered to include the scaffold protein ISCU,cysteine desulfurase NFS1 and its accessory pro-teins ISD11 and ACP,and frataxin (FXN).Proteins involved in Fe-S cluster transfer have been found to include HSC20/HSPA9,as chaperone system,and Fe-S cluster carriers.The biosynthesis and transfer of Fe-S clusters to Fe-S recipients require fine-tune regulation.Recently,significant progress has been made in the structure and mechanism of mitochondrial Fe-S biosynthesis and transfer.Based on,espe-cially,the development of DNA sequencing technology,bioinformatics,and gene editing technology,diseases caused by mutations of Fe-S cluster-related genes have been revealed in recent years,promot-ing the rapid development in the field of Fe-S and human health.This review focuses on the function of genes involved in Fe-S cluster biosynthesis and transfer and on the diseases caused by the mutations of the related genes.Finally,some questions we are facing are raised,new hypotheses presented,and the perspectives discussed.
MitochondriaFe-S cluster synthesis and transferCongenital sideroblastic anemiaNeurodegenerative diseases
Wenxin Zhang、Li Xu、Hongting Zhao、Kuanyu Li
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Medical School of Nanjing University,Jiangsu Key Laboratory of Molecular Medicine,Nanjing 210093,China
This work was supported by the National Natural Science foundation of China
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