Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion

Victoria Campuzano, Laura Montermini, María Dolores Moltó, +23

Science

Mechanisms of Cell Death in Oxidative Stress

Stefan W. Ryter, Hong Pyo Kim, Alexander Hoetzel, +4

Antioxidants and Redox Signaling

Aconitase and mitochondrial iron–sulphur protein deficiency in Friedreich ataxia

Agnès Rötig, Pascale de Lonlay, Dominique Chrétien, +4

Nature Genetics

Mitochondrial FunctionBiochemistryIron MetabolismDegenerative DiseaseFriedreich Ataxia +4

Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin

Michael Babcock, Deepika de Silva, Robert Oaks, +5

Science

Iron MetabolismGeneticsPutative HomologMolecular BiologyMolecular Genetics +17

Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin

Hana Koutníková, Victoria Campuzano, Françoise Foury, +2

Nature Genetics

BiologyMitophagyMitochondrial FunctionOrganelle DynamicNatural Sciences +10

Inactivation-reactivation of aconitase in Escherichia coli. A sensitive measure of superoxide radical.

Paul R. Gardner, Irwin Fridovich

Journal of Biological Chemistry

Cellular IAPs inhibit a cryptic CD95-induced cell death by limiting RIP1 kinase recruitment

Peter Geserick, Mike Hupe, Maryline Moulin, +6

The Journal of Cell Biology

Induction of Oxidative Metabolism by Mitochondrial Frataxin Inhibits Cancer Growth

Tim J. Schulz, René Thierbach, Anja Voigt, +5

Journal of Biological Chemistry

Oxidative MetabolismCell DeathOtto WarburgRedox BiologyTumor Biology +14

The in vivo mitochondrial two-step maturation of human frataxin

Stéphane Schmucker, Manuela Argentini, Nadège Calmels, +2

Human Molecular Genetics

Amino AcidsMolecular BiologyHuman FrataxinRedox BiologyFriedreich Ataxia +15

Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice

René Thierbach, Tim J. Schulz, Frank Isken, +10

Human Molecular Genetics