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FARAFARA Cure FA

Molecular Details of the Yeast Frataxin-Isu1 Interaction during Mitochondrial Fe-S Cluster Assembly

Frataxin, a conserved nuclear-encoded mitochondrial protein, plays a direct role in iron−sulfur cluster biosynthesis within the ISC assembly pathway. Humans with frataxin deficiency have Friedreich’s ataxia, a neurodegenerative disorder characterized by mitochondrial iron overload and disruption in Fe−S cluster synthesis. Biochemical and genetic studies have shown frataxin interacts with the iron−sulfur cluster assembly scaffold protein (in yeast, there are two, Isu1 and Isu2), indicating frataxin plays a direct role in cluster assembly, possibly by serving as an iron chaperone in the assembly pathway.

Molecular Details of the Yeast Frataxin-Isu1 Interaction during Mitochondrial Fe-S Cluster Assembly


About the Author

Jen Farmer

Jen Farmer

Executive Director

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