Frataxin Silencing Inactivates Mitochondrial Complex I in NSC34 Motoneuronal Cells and Alters Glutathione Homeostasis

Complex I and ATP Content Deficiency in Lymphocytes from Friedreich's Ataxia

(2014) Mohammad Mehdi Heidari et al.   CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES

Generation and Characterisation of Friedreich Ataxia YG8R Mouse Fibroblast and Neural Stem Cell Models

(2014) Chiranjeevi Sandi et al.   PLoS One

Frataxin Deficiency Leads to Defects in Expression of Antioxidants and Nrf2 Expression in Dorsal Root Ganglia of the Friedreich's Ataxia YG8R Mouse Model

(2013) Yuxi Shan et al.   ANTIOXIDANTS & REDOX SIGNALING

Frataxin Deficiency Leads to Reduced Expression and Impaired Translocation of NF-E2-Related Factor (Nrf2) in Cultured Motor Neurons

(2013) Valentina D'Oria et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Induced Pluripotent Stem Cells from Friedreich Ataxia Patients Fail to Upregulate Frataxin During In Vitro Differentiation to Peripheral Sensory Neurons

(2013) Andreas Eigentler et al.   STEM CELLS AND DEVELOPMENT

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

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

S-Glutathionylation signaling in cell biology: Progress and prospects

(2012) Anna Pastore et al.   EUROPEAN JOURNAL OF PHARMACEUTICAL SCIENCES

A gene expression phenotype in lymphocytes from friedreich ataxia patients

(2011) Giovanni Coppola et al.   ANNALS OF NEUROLOGY

Mechanisms of Altered Redox Regulation in Neurodegenerative Diseases—Focus on S-Glutathionylation

(2011) Elizabeth A. Sabens Liedhegner et al.   ANTIOXIDANTS & REDOX SIGNALING

Posttranslational Modification of Cysteine in Redox Signaling and Oxidative Stress: Focus on S-Glutathionylation

(2011) John J. Mieyal et al.   ANTIOXIDANTS & REDOX SIGNALING

HSC20 interacts with frataxin and is involved in iron–sulfur cluster biogenesis and iron homeostasis

(2011) Yuxi Shan et al.   HUMAN MOLECULAR GENETICS

Silencing of frataxin gene expression triggers p53-dependent apoptosis in human neuron-like cells

(2011) G. M. Palomo et al.   HUMAN MOLECULAR GENETICS

Effect of protein glutathionylation on neuronal cytoskeleton: a potential link to neurodegeneration

(2011) B. Carletti et al.   NEUROSCIENCE

Mammalian Frataxin: An Essential Function for Cellular Viability through an Interaction with a Preformed ISCU/NFS1/ISD11 Iron-Sulfur Assembly Complex

(2011) Stéphane Schmucker et al.   PLoS One

Friedreich Ataxia: Molecular Mechanisms, Redox Considerations, and Therapeutic Opportunities

(2010) Renata Santos et al.   ANTIOXIDANTS & REDOX SIGNALING

Friedreich's Ataxia Induced Pluripotent Stem Cells Model Intergenerational GAA⋅TTC Triplet Repeat Instability

(2010) Sherman Ku et al.   Cell Stem Cell

Does oxidative stress contribute to the pathology of Friedreich’s ataxia? A radical question

(2010) Jeffrey S. Armstrong et al.   FASEB JOURNAL

Generation of Induced Pluripotent Stem Cell Lines from Friedreich Ataxia Patients

(2010) Jun Liu et al.   Stem Cell Reviews and Reports

Altered Gene Expression and DNA Damage in Peripheral Blood Cells from Friedreich's Ataxia Patients: Cellular Model of Pathology

(2010) Astrid C. Haugen et al.   PLoS Genetics

Frataxin deficiency induces Schwann cell inflammation and death

(2009) Chunye Lu et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARγ pathway as a therapeutic target in Friedreich’s ataxia

(2009) Giovanni Coppola et al.   HUMAN MOLECULAR GENETICS

The ins and outs of mitochondrial iron-loading: the metabolic defect in Friedreich’s ataxia

(2009) Des R. Richardson et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Friedreich's ataxia: Oxidative stress and cytoskeletal abnormalities

(2009) Marco Sparaco et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Diagnosis and treatment of Friedreich ataxia: a European perspective

(2009) Jörg B. Schulz et al.   Nature Reviews Neurology

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

(2009) Nadège Calmels et al.   PLoS One

The Nrf2/ARE Pathway as a Potential Therapeutic Target in Neurodegenerative Disease

(2008) Marcus J. Calkins et al.   ANTIOXIDANTS & REDOX SIGNALING

Mammalian complex I: A regulable and vulnerable pacemaker in mitochondrial respiratory function

(2008) Sergio Papa et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Glutathione-dependent redox status of frataxin-deficient cells in a yeast model of Friedreich's ataxia

(2008) F. Auchere et al.   HUMAN MOLECULAR GENETICS

Long intronic GAA•TTC repeats induce epigenetic changes and reporter gene silencing in a molecular model of Friedreich ataxia

(2008) E. Soragni et al.   NUCLEIC ACIDS RESEARCH

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