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

Multicellular models of Friedreich ataxia

(2009) Hélène Puccio   JOURNAL OF NEUROLOGY

The pathogenesis of Friedreich ataxia and the structure and function of frataxin

(2009) Massimo Pandolfo et al.   JOURNAL OF NEUROLOGY

Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS

(2009) Salvatore Adinolfi et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Impaired Nuclear Nrf2 Translocation Undermines the Oxidative Stress Response in Friedreich Ataxia

(2009) Vincent Paupe et al.   PLoS One

Dynamics, stability and iron-binding activity of frataxin clinical mutants

(2008) Ana R. Correia et al.   FEBS Journal

Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia

(2008) P. R. Anderson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The effects of frataxin silencing in HeLa cells are rescued by the expression of human mitochondrial ferritin

(2007) Isabella Zanella et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE