A multiple animal and cellular models approach to study frataxin deficiency in Friedreich Ataxia von Mosbach, Valentine, Puccio, Hélène

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Mammalian Fe-S cluster biogenesis and its implication in disease von Beilschmidt, Lena K, Puccio, Hélène M

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Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex von Schmucker, Stéphane, Martelli, Alain, Colin, Florent, Page, Adeline, Wattenhofer-Donzé, Marie, Reutenauer, Laurence, Puccio, Hélène

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Mammalian Frataxin Controls Sulfur Production and Iron Entry during de Novo Fe4S4 Cluster Assembly von Colin, Florent, Martelli, Alain, Clémancey, Martin, Latour, Jean-Marc, Gambarelli, Serge, Zeppieri, Laura, Birck, Catherine, Page, Adeline, Puccio, Hélène, Ollagnier de Choudens, Sandrine

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Autosomal Recessive Cerebellar Ataxias: Paving the Way toward Targeted Molecular Therapies von Synofzik, Matthis, Puccio, Hélène, Mochel, Fanny, Schöls, Ludger

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Correction of half the cardiomyocytes fully rescue Friedreich ataxia mitochondrial cardiomyopathy through cell-autonomous mechanisms von Belbellaa, Brahim, Reutenauer, Laurence, Monassier, Laurent, Puccio, Hélène

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Omaveloxolone: a groundbreaking milestone as the first FDA-approved drug for Friedreich ataxia von Pilotto, Federica, Chellapandi, Deepika M., Puccio, Hélène

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Primary Cultures of Pure Embryonic Dorsal Root Ganglia Sensory Neurons as a New Cellular Model for Friedreich’s Ataxia von Griso, Olivier, Puccio, Hélène

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Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia von Piguet, Françoise, de Montigny, Charline, Vaucamps, Nadège, Reutenauer, Laurence, Eisenmann, Aurélie, Puccio, Hélène

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Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia von Perdomini, Morgane, Belbellaa, Brahim, Monassier, Laurent, Reutenauer, Laurence, Messaddeq, Nadia, Cartier, Nathalie, Crystal, Ronald G, Aubourg, Patrick, Puccio, Hélène

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Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency von Sayles, Nicole M, Napierala, Jill S, Anrather, Josef, Diedhiou, Nadège, Li, Jixue, Napierala, Marek, Puccio, Hélène, Manfredi, Giovanni

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Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation von Martelli, Alain, Puccio, Hélène

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High Levels of Frataxin Overexpression Lead to Mitochondrial and Cardiac Toxicity in Mouse Models von Belbellaa, Brahim, Reutenauer, Laurence, Messaddeq, Nadia, Monassier, Laurent, Puccio, Hélène

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Hereditary Ataxias: From Bench to Clinic, Where Do We Stand? von Pilotto, Federica, Del Bondio, Andrea, Puccio, Hélène

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Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches von Schmucker, Stéphane, Puccio, Hélène

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ISCA1 is essential for mitochondrial Fe4S4 biogenesis in vivo von Beilschmidt, Lena Kristina, Ollagnier de Choudens, Sandrine, Fournier, Marjorie, Sanakis, Ioannis, Hograindleur, Marc-André, Clémancey, Martin, Blondin, Geneviève, Schmucker, Stéphane, Eisenmann, Aurélie, Weiss, Amélie, Koebel, Pascale, Messaddeq, Nadia, Puccio, Hélène, Martelli, Alain

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Mitochondrial dysfunction and calcium dysregulation in COQ8A-ataxia Purkinje neurons are rescued by CoQ10 treatment von Manolaras, Ioannis, Del Bondio, Andrea, Griso, Olivier, Reutenauer, Laurence, Eisenmann, Aurélie, Habermann, Bianca H, Puccio, Hélène

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Red blood cell generation from human induced pluripotent stem cells: perspectives for transfusion medicine von LAPILLONNE, Hélène, KOBARI, Ladan, FRANCINA, Alain, ANDREU, Georges, VIVILLE, Stéphane, DOUAY, Luc, MAZURIER, Christelle, TROPEL, Philippe, GIARRATANA, Marie-Catherine, ZANELLA-CLEON, Isabelle, KIGER, Laurent, WATTENHOFER-DONZE, Marie, PUCCIO, Hélène, HEBERT, Nicolas

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Animal and cellular models of Friedreich ataxia von Perdomini, Morgane, Hick, Aurore, Puccio, Hélène, Pook, Mark A.

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Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular models von Martelli, Alain, Napierala, Marek, Puccio, Hélène

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