Hélène PUCCIO
Researcher
Publications
2011
Article in a journal
Mammalian Frataxin: An Essential Function for Cellular Viability through an Interaction with a Preformed ISCU/NFS1/ISD11 Iron-Sulfur Assembly Complex
- Stephane Schmucker
- Alain Martelli
- Florent Colin
- Adeline Page
- Marie Wattenhofer-Donzé
- Laurence Reutenauer
- Hélène Puccio
PLoS ONE ; Volume: 6 ; Page: e16199
2010
Article in a journal
Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches
- Stephane Schmucker
- Hélène Puccio
Human Molecular Genetics ; Volume: 19 ; Page: R103-110
2009
Article in a journal
Multicellular models of Friedreich ataxia.
- Hélène Puccio
Deutsche Zeitschrift für Nervenheilkunde / Deutsche Zeitschrift f ur Nervenheilkunde ; Volume: 256 Suppl 1 ; Page: 18-24
Article in a journal
The first cellular models based on frataxin missense mutations that reproduce spontaneously the defects associated with Friedreich ataxia.
- Nadège Calmels
- Stéphane Schmucker
- Marie Wattenhofer-Donzé
- Alain Martelli
- Nadège Vaucamps
- Laurence Reutenauer
- Nadia Messaddeq
- Cécile Bouton
- Michel Koenig
- Hélène Puccio
PLoS ONE ; Volume: 4 ; Page: e6379
Article in a journal
Limitations in a frataxin knockdown cell model for Friedreich ataxia in a high-throughput drug screen.
- Nadege Calmels
- Hervé Seznec
- Pascal Villa
- Laurence Reutenauer
- Marcel Hibert
- Jacques Haeich
- Pierre Rustin
- Michel Koenig
- Hélène Puccio
- Hélène Puccio
BMC Neurology ; Volume: 9 ; Page: 46
2008
Article in a journal
The in vivo mitochondrial two-step maturation of human frataxin.
- Stéphane Schmucker
- Manuela Argentini
- Nadège Carelle-Calmels
- Alain Martelli
- Hélène Puccio
Human Molecular Genetics ; Volume: 17 ; Page: 3521-31
2007
Chapter of the book
Conditional mouse models for Friedreich ataxia, a neurodegenerative disorder associating cardiomyopathy.
- Hélène Puccio
Handbook of Experimental Pharmacology ; Page: 365-75
Article in a journal
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
Molecular Genetics and Metabolism ; Volume: 92 ; Page: 23-35
2006
Chapter of the book
Mouse Models for Friedreich's Ataxia
- Hélène Puccio
Genetic Instabilities and Neurological Diseases (Second Edition) ; Page: 321-326
2005
Article in a journal
Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells.
- Robert A Schoenfeld
- Eleonora Napoli
- Alice Wong
- Shan Zhan
- Laurence Reutenauer
- Dexter Morin
- Alan R Buckpitt
- Franco Taroni
- Bo Lonnerdal
- Michael Ristow
- Hélène Puccio
- Gino A Cortopassi
Human Molecular Genetics ; Volume: 14 ; Page: 3787-99