Mammalian Frataxin: An Essential Function for Cellular Viability through an Interaction with a Preformed ISCU/NFS1/ISD11 Iron-Sulfur Assembly Complex

PLoS ONE 6(1): e16199. doi:10.1371/journal.pone.0016199

Stéphane Schmucker1,2,3,4,5, Alain Martelli1,2,3,4,5, Florent Colin1,2,3,4,5, Adeline Page1,2,3,4, Marie Wattenhofer-Donzé1,2,3,4,5, Laurence Reutenauer1,2,3,4,5, Hélène Puccio1,2,3,4,5*

1 Department of Translational Medicine and Neurogenetics, Institut de Génétique et de Biologie Moléculaire et Cellulaire (IGBMC), Illkirch, France, 2 Inserm U596, Illkirch, France, 3 CNRS UMR7104, Illkirch, France, 4 Université de Strasbourg, Strasbourg, France, 5 Chaire de Génétique Humaine, Collège de France, Illkirch, France

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Conclusions/Significance

Our results suggest that the interaction of frataxin with the core ISCU/NFS1/ISD11 complex most likely defines the essential function of frataxin. Our results provide new elements important for further understanding the early steps of de novo Fe-S cluster biosynthesis

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