The role of oxidative stress in Friedreich's ataxia

Oxidative Stress: Mechanistic Insights into Inherited Mitochondrial Disorders and Parkinson’s Disease

(2017) Mesfer Al Shahrani et al.   Journal of Clinical Medicine

Lentivirus-meditated frataxin gene delivery reverses genome instability in Friedreich ataxia patient and mouse model fibroblasts

(2016) H Khonsari et al.   GENE THERAPY

Deletion of the GAA repeats from the human frataxin gene using the CRISPR-Cas9 system in YG8R-derived cells and mouse models of Friedreich ataxia

(2016) D L Ouellet et al.   GENE THERAPY

'Mitochondrial energy imbalance and lipid peroxidation cause cell death in Friedreich’s ataxia'

(2016) R Abeti et al.   Cell Death & Disease

Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration

(2016) Kuchuan Chen et al.   eLife

Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammals

(2016) Kuchuan Chen et al.   eLife

Time-resolved functional analysis of acute impairment offrataxinexpression in an inducible cell model of Friedreich ataxia

(2016) Dörte Poburski et al.   Biology Open

A new cellular model to follow Friedreich's ataxia development in a time-resolved way

(2015) T. Vannocci et al.   Disease Models & Mechanisms

A novel GAA-repeat-expansion-based mouse model of Friedreich's ataxia

(2015) S. Anjomani Virmouni et al.   Disease Models & Mechanisms

TORC1 Inhibition by Rapamycin Promotes Antioxidant Defences in a Drosophila Model of Friedreich’s Ataxia

(2015) Pablo Calap-Quintana et al.   PLoS One

FXN Promoter Silencing in the Humanized Mouse Model of Friedreich Ataxia

(2015) Yogesh K. Chutake et al.   PLoS One

Real-Time Metabolic Analysis of Living Cancer Cells with Correlated Cellular Spectro-microscopy

(2014) Luca Quaroni et al.   ANALYTICAL CHEMISTRY

Real-Time Monitoring of Protein Conformational Changes Using a Nano-Mechanical Sensor

(2014) Livan Alonso-Sarduy et al.   PLoS One

Detecting nanoscale vibrations as signature of life

(2014) Sandor Kasas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Pathophysiogical and therapeutic progress in Friedreich ataxia

(2014) H. Puccio et al.   REVUE NEUROLOGIQUE

Chronochemistry in neurodegeneration

(2014) Annalisa Pastore et al.   Frontiers in Molecular Neuroscience

Metabolic remodeling in frataxin-deficient yeast is mediated by Cth2 and Adr1

(2013) Armando Moreno-Cermeño et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Deferiprone and idebenone rescue frataxin depletion phenotypes in a Drosophila model of Friedreich's ataxia

(2013) Sirena Soriano et al.   GENE

Frataxin: a protein in search for a function

(2013) Annalisa Pastore et al.   JOURNAL OF NEUROCHEMISTRY

Rapid detection of bacterial resistance to antibiotics using AFM cantilevers as nanomechanical sensors

(2013) G. Longo et al.   Nature Nanotechnology

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

(2012) A. Martelli et al.   Disease Models & Mechanisms

Estrogen Prevents Oxidative Damage to the Mitochondria in Friedreich's Ataxia Skin Fibroblasts

(2012) Timothy E. Richardson et al.   PLoS One

Expansion of Intronic GGCCTG Hexanucleotide Repeat in NOP56 Causes SCA36, a Type of Spinocerebellar Ataxia Accompanied by Motor Neuron Involvement

(2011) Hatasu Kobayashi et al.   AMERICAN JOURNAL OF HUMAN GENETICS

TALEs of genome targeting

(2011) Jens Boch   NATURE BIOTECHNOLOGY

Fluorescent Protein-Based Redox Probes

(2010) Andreas J. Meyer et al.   ANTIOXIDANTS & REDOX SIGNALING

Altered lipid metabolism in a Drosophila model of Friedreich's ataxia

(2010) J. A. Navarro et al.   HUMAN MOLECULAR GENETICS

Frataxin Depletion in Yeast Triggers Up-regulation of Iron Transport Systems before Affecting Iron-Sulfur Enzyme Activities

(2010) Armando Moreno-Cermeño et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Defects in Mitochondrial Axonal Transport and Membrane Potential without Increased Reactive Oxygen Species Production in a Drosophila Model of Friedreich Ataxia

(2010) Y. Shidara et al.   JOURNAL OF NEUROSCIENCE

Genome editing with engineered zinc finger nucleases

(2010) Fyodor D. Urnov et al.   NATURE REVIEWS GENETICS

Structural bases for the interaction of frataxin with the central components of iron–sulphur cluster assembly

(2010) Filippo Prischi et al.   Nature Communications

Friedreich ataxia: The clinical picture

(2009) Massimo Pandolfo   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

Major targets of iron-induced protein oxidative damage in frataxin-deficient yeasts are magnesium-binding proteins

(2008) Verónica Irazusta et al.   FREE RADICAL BIOLOGY AND MEDICINE

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