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1

Mammalian Frataxin Controls Sulfur Production and Iron Entry during de Novo Fe 4 S 4 Cluster Assembly

Colin, Florent, Martelli, Alain, Clémancey, Martin, Latour, Jean-Marc, Gambarelli, Serge, Zeppieri, Laura, Birck, Catherine, Page, Adeline, Puccio, Hélène, Ollagnier de Choudens, Sandrine

Journal:

Journal of the American Chemical Society

Year:

2013

Language:

english

File:

PDF, 395 KB

english, 2013

2

Friedreich ataxia: a paradigm for mitochondrial diseases

Hélène Puccio, Michel Kœnig

Journal:

Current Opinion in Genetics & Development

Year:

2002

Language:

english

File:

PDF, 63 KB

english, 2002

3

ChemInform Abstract: Understanding the Molecular Mechanisms of Friedreich′s Ataxia to Develop Therapeutic Approaches

Stephane Schmucker, Helene Puccio

Journal:

Year:

2010

File:

PDF, 15 KB

2010

4

Multicellular models of Friedreich ataxia

Hélène Puccio

Journal:

Journal of Neurology

Year:

2009

Language:

english

File:

PDF, 170 KB

english, 2009

5

The genomic organization of human dystrobrevin

Hélène M. Sadoulet-Puccio, Chris A. Feener, Daniel J. Schaid, Stephen N. Thibodeau, Virginia V. Michels, Louis M. Kunkel

Journal:

Year:

1997

Language:

english

File:

PDF, 202 KB

english, 1997

6

Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesis

Mark A. Pook, Sahar Al-Mahdawi, Christopher J. Carroll, Mireille Cossée, Hélène Puccio, Lorraine Lawrence, Peter Clark, Margaret B. Lowrie, Jane L. Bradley, Mark J. Cooper, Michel Kœnig, Susan Chamber

Journal:

Year:

2001

Language:

english

File:

PDF, 258 KB

english, 2001

7

Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe–S proteins

Blanche Guillon, Anne-Laure Bulteau, Marie Wattenhofer-Donzé, Stéphane Schmucker, Bertrand Friguet, Hélène Puccio, Jean-Claude Drapier, Cécile Bouton

Journal:

Year:

2009

Language:

english

File:

PDF, 412 KB

english, 2009

8

The molecular pathogenesis of Friedreich ataxia

Hélène Puccio, Michel Kœnig

Journal:

Clinical Neuroscience Research

Year:

2001

Language:

english

File:

PDF, 157 KB

english, 2001

9

Limitations in a frataxin knockdown cell model for Friedreich ataxia in a high-throughput drug screen

Nadège Calmels, Hervé Seznec, Pascal Villa, Laurence Reutenauer, Marcel Hibert, Jacques Haiech, Pierre Rustin, Michel Koenig, Hélène Puccio

Journal:

Year:

2009

Language:

english

File:

PDF, 1.27 MB

english, 2009

10

2003 International Friedreich's Ataxia Research Conference, 14–16 February 2003, Bethesda, MD, USA

Hervé Seznec, Robert B Wilson, Hélène Puccio

Journal:

Neuromuscular Disorders

Year:

2004

Language:

english

File:

PDF, 156 KB

english, 2004

11

Advancements in the pathophysiology of Friedreich’s Ataxia and new prospects for treatments

Ngolela E. Babady, Nadege Carelle, Robert D. Wells, Tracey A. Rouault, Michio Hirano, David R. Lynch, Martin B. Delatycki, Robert B. Wilson, Grazia Isaya, Hélène Puccio

Journal:

Molecular Genetics and Metabolism

Year:

2007

Language:

english

File:

PDF, 210 KB

english, 2007

12

10.1038/84818

Puccio, Hélène, Simon, Delphine, Cossée, Mireille, Criqui-Filipe, Paola, Tiziano, Francesco, Melki, Judith, Hindelang, Colette, Matyas, Robert, Rustin, Pierre, Koenig, Michel

Journal:

Nature Genetics

Year:

2001

Language:

english

File:

PDF, 3.08 MB

english, 2001

13

Mammalian Fe–S cluster biogenesis and its implication in disease

Beilschmidt, Lena K., Puccio, Hélène M.

Journal:

Year:

2014

Language:

english

File:

PDF, 1011 KB

english, 2014

14

Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia

Perdomini, Morgane, Belbellaa, Brahim, Monassier, Laurent, Reutenauer, Laurence, Messaddeq, Nadia, Cartier, Nathalie, Crystal, Ronald G, Aubourg, Patrick, Puccio, Hélène

Journal:

Nature Medicine

Year:

2014

Language:

english

File:

PDF, 1.63 MB

english, 2014

15

The molecular genetics of coenzyme Q biosynthesis in health and disease

Laredj, Leila N., Licitra, Floriana, Puccio, Hélène M.

Journal:

Year:

2014

Language:

english

File:

PDF, 665 KB

english, 2014

16

Dystrobrevin and Dystrophin: An Interaction through Coiled-Coil Motifs

Helene M. Sadoulet-Puccio, Michael Rajala and Louis M. Kunkel

Journal:

Proceedings of the National Academy of Sciences

Year:

1997

Language:

english

File:

PDF, 1.51 MB

english, 1997

17

Differential Membrane Localization and Intermolecular Associations of α-Dystrobrevin Isoforms in Skeletal Muscle

Matthew F. Peters, Hélène M. Sadoulet-Puccio, R. Mark Grady, Neal R. Kramarcy, Louis M. Kunkel, Joshua R. Sanes, Robert Sealock and Stanley C. Froehner

Journal:

The Journal of Cell Biology

Year:

1998

Language:

english

File:

PDF, 2.54 MB

english, 1998

18

0300: Systemic delivery of AAVrh10 expressing frataxin corrects the severe mitochondrial cardiomyopathy of frataxin deficient mice

Belbellaa, Brahim, Perdomini, Morgane, Monassier, Laurent, Aubourg, Patrick, Puccio, Hélène

Journal:

Archives of Cardiovascular Diseases Supplements

Year:

2014

Language:

english

File:

PDF, 50 KB

english, 2014

19

Frataxin: a protein in search for a function

Pastore, Annalisa, Puccio, Helene

Journal:

Journal of Neurochemistry

Year:

2013

Language:

english

File:

PDF, 341 KB

english, 2013

20

Animal and cellular models of Friedreich ataxia

Perdomini, Morgane, Hick, Aurore, Puccio, Hélène, Pook, Mark A.

Journal:

Journal of Neurochemistry

Year:

2013

Language:

english

File:

PDF, 283 KB

english, 2013

21

An Overview of Current Mouse Models Recapitulating Coenzyme Q10 Deficiency Syndrome

Licitra, Floriana, Puccio, Hélène

Journal:

Molecular Syndromology

Year:

2014

Language:

english

File:

PDF, 405 KB

english, 2014

22

Iron Regulatory Protein 1 Sustains Mitochondrial Iron Loading and Function in Frataxin Deficiency

Martelli, Alain, Schmucker, Stéphane, Reutenauer, Laurence, Mathieu, Jacques R.R., Peyssonnaux, Carole, Karim, Zoubida, Puy, Hervé, Galy, Bruno, Hentze, Matthias W., Puccio, Hélène

Journal:

Cell Metabolism

Year:

2015

Language:

english

File:

PDF, 3.27 MB

english, 2015

23

Creativity on demand: Historical approaches and future trends

Puccio, Gerard J., Cabra, John F., Fox, J. Michael, Cahen, Helene

Journal:

Artificial intelligence for engineering design analysis and manufacturing

Year:

2010

Language:

english

File:

PDF, 228 KB

english, 2010

24

54. Prevention of Sensory Ataxia in a Novel Mouse Model of Friedreich Ataxia Using Gene Therapy Approach

Piguet, Françoise, de Montigny, Charline, Vaucamps, Nadège, Reutenauer, Laurence, Puccio, Hélène

Journal:

Molecular Therapy

Year:

2016

Language:

english

File:

PDF, 299 KB

english, 2016

25

Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia

Piguet, Françoise, de Montigny, Charline, Vaucamps, Nadège, Reutenauer, Laurence, Eisenmann, Aurélie, Puccio, Hélène

Journal:

Molecular Therapy

Year:

2018

Language:

english

File:

PDF, 4.89 MB

english, 2018

26

The First Cellular Models Based on Frataxin Missense Mutations That Reproduce Spontaneously the Defects Associated with Friedreich Ataxia

Calmels, Nadège, Schmucker, Stéphane, Wattenhofer-Donzé, Marie, Martelli, Alain, Vaucamps, Nadège, Reutenauer, Laurence, Messaddeq, Nadia, Bouton, Cécile, Koenig, Michel, Puccio, Hélène, Nollen, Ellen

Journal:

Year:

2009

Language:

english

File:

PDF, 594 KB

english, 2009

27

Vers une thérapie génique pour la cardiomyopathie associée à l’ataxie de Friedreich

Belbellaa, Brahim, Puccio, Hélène

Journal:

médecine/sciences

Year:

2014

Language:

french

File:

PDF, 1.04 MB

french, 2014

28

Correction of half the cardiomyocytes fully rescue Friedreich Ataxia mitochondrial cardiomyopathy through cell-autonomous mechanisms

Belbellaa, Brahim, Reutenauer, Laurence, Monassier, Laurent, Puccio, Hélène

Journal:

Human Molecular Genetics

Year:

2018

Language:

english

File:

PDF, 7.30 MB

english, 2018