Hélène PUCCIO

Publications

• 2014

  • Article in a journal

    The Diabetes Drug Target MitoNEET Governs a Novel Trafficking Pathway to Rebuild an Fe-S Cluster into Cytosolic Aconitase/Iron Regulatory Protein 1

    • Ioana Ferecatu
    • Sergio Gonçalves
    • Marie-Pierre Golinelli-Cohen
    • Martin Clémancey
    • Alain Martelli
    • Sylvie Riquier
    • Eric Guittet
    • Jean-Marc Latour
    • Hélène Puccio
    • Jean-Claude Drapier
    • Ewen Lescop
    • Cécile Bouton

    Journal of Biological Chemistry ; Volume: 289 ; Page: 28070 - 28086

    HALDOI

  • Article in a journal

    Mammalian Fe–S cluster biogenesis and its implication in disease

    • Lena Beilschmidt
    • Hélène Puccio

    Biochimie ; Volume: 100 ; Page: 48-60

    HALDOI

  • Article in a journal

    Pathophysiogical and therapeutic progress in Friedreich ataxia

    • Hélène Puccio
    • Mathieu Anheim
    • Christine Tranchant

    Revue Neurologique ; Volume: 170 ; Page: 355-365

    HALDOI

  • Article in a journal  » Review article

    Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation

    • Alain Martelli
    • Hélène Puccio

    Frontiers in Pharmacology ; Volume: 5 ; Page: 130

    HALDOI

  • Article in a journal

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

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

    Nature Medicine ; Volume: 20 ; Page: 542--547

    HALDOI

• 2013

  • Article in a journal

    Animal and cellular models of Friedreich ataxia

    • Morgane Perdomini
    • Aurore Hick
    • Hélène Puccio
    • Mark Pook

    Journal of Neurochemistry ; Volume: 126 ; Page: 65-79

    HALDOI

  • Article in a journal

    Neurons and cardiomyocytes derived from induced pluripotent stem cells as a model for mitochondrial defects in Friedreich's ataxia

    • Aurore Hick
    • Marie Wattenhofer-Donzé
    • Satyan Chintawar
    • Philippe Tropel
    • Jodie Simard
    • Nadège Vaucamps
    • David Gall
    • Laurie Lambot
    • Cécile André
    • Laurence Reutenauer
    • Myriam Rai
    • Marius Teletin
    • Nadia Messaddeq
    • Serge Schiffmann
    • Stéphane Viville
    • Christopher Pearson
    • Massimo Pandolfo
    • Hélène Puccio

    Disease Models & Mechanisms ; Volume: 6 ; Page: 608-621

    HALDOI

• 2012

  • Article in a journal

    Mammalian frataxin controls sulfur production and iron entry during de novo Fe4S4 cluster assembly.

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

    Journal of the American Chemical Society ; Volume: 135 ; Page: 733-40

    HALDOI

  • Article in a journal

    Clinical data and characterization of the liver conditional mouse model exclude neoplasia as a non-neurological manifestation associated with Friedreich's ataxia

    • Alain Martelli
    • Lisa S. Friedman
    • Laurence Reutenauer
    • Nadia Messaddeq
    • Susan L. Perlman
    • David R. Lynch
    • Kathrin Fedosov
    • Jörg B Schulz
    • Massimo Pandolfo
    • Hélène Puccio

    Disease Models & Mechanisms ; Volume: 5 ; Page: 860–869

    HALDOI

  • Article in a journal  » Review article

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

    • Alain Martelli
    • Marek Napierala
    • Hélène Puccio

    Disease Models & Mechanisms ; Volume: 5 ; Page: 165 - 176

    HALDOI