作者
Timothy L Stemmler, Emmanuel Lesuisse, Debkumar Pain, Andrew Dancis
发表日期
2010/8/27
期刊
Journal of Biological Chemistry
卷号
285
期号
35
页码范围
26737-26743
出版商
American Society for Biochemistry and Molecular Biology
简介
Friedreich ataxia is an inherited neurodegenerative disease caused by frataxin deficiency. Frataxin is a conserved mitochondrial protein that plays a role in FeS cluster assembly in mitochondria. FeS clusters are modular cofactors that perform essential functions throughout the cell. They are synthesized by a multistep and multisubunit mitochondrial machinery that includes the scaffold protein Isu for assembling a protein-bound FeS cluster intermediate. Frataxin interacts with Isu, iron, and the cysteine desulfurase Nfs1, which supplies sulfide, thus placing it at the center of mitochondrial FeS cluster biosynthesis.
学术搜索中的文章
TL Stemmler, E Lesuisse, D Pain, A Dancis - Journal of Biological Chemistry, 2010