Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease

Dis. Model. Mech. March 2012, doi: 10.1242/dmm.009019 vol. 5 no. 2 155-164

Tracey A. Rouault
National Institute of Child Health and Human Development, Bethesda, MD

Keywords: Iron-sulfur (Fe-S) clusters, electron transport, respiratory chain complexes, regulatory sensing, photosynthesis, DNA repair, Friedreich’s ataxia (FRDA), ISCU myopathy, sideroblastic anemia, encephalomyopathy, mitochondrial iron overload.

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Understanding the genetic and molecular pathogenesis of Friedreich’s ataxia through animal and cellular models

Dis. Model. Mech. March 2012, doi: 10.1242/dmm.008706 vol. 5 no. 2 165-176

Alain Martelli 1,2,3,4,5, Marek Napierala 6 and Hélène Puccio 1,2,3,4,5,*
1 Translational Medicine and Neurogenetics, IGBMC (Institut de Génétique et de Biologie Moléculaire et Cellulaire), 67404, Illkirch, France
2 Inserm, U596, 67400, Illkirch, France
3 CNRS, UMR7104, 67400, Illkirch, France
4 Université de Strasbourg, 67000, Strasbourg, France
5 Collège de France, Chaire de génétique humaine, 67400, Illkirch, France
6 The Department of Molecular Carcinogenesis, Center for Cancer Epigenetics, University of Texas MD Anderson Cancer Center Science Park, Smithville, TX, USA

Keywords: Friedreich’s ataxia (FRDA), frataxin (FXN), GAA.

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