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Ataxia

New ataxia study findings reported from A. Martelli and co-authors

Published in Blood Weekly, December 13th, 2007

Research findings, 'Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues,' are discussed in a new report. "Friedreich ataxia, the most common recessive ataxia, is caused by the deficiency of the mitochondrial protein frataxin (Fxn), an iron chaperone involved in the assembly of Fe-S clusters (ISC). In yeast, mitochondria play a central role for all Fe-S proteins, independently of their subcellular localization," investigators in Illkirch, France report.

"In mammalian cells, this central role of mitochondria remains controversial as an independent cytosolic ISC assembly machinery has been suggested. In the present work, we show that...

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