期刊
JOURNAL OF BIOLOGICAL CHEMISTRY
卷 285, 期 35, 页码 26737-26743出版社
AMER SOC BIOCHEMISTRY MOLECULAR BIOLOGY INC
DOI: 10.1074/jbc.R110.118679
关键词
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资金
- National Institutes of Health [DK068139, AG030504, R37DK053953]
- Association Francaise de l'Ataxie de Friedreich
- American Heart Association [09GRNT2260364]
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.
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