PPAR gamma agonist leriglitazone improves frataxin-loss impairments in cellular and animal models of Friedreich Ataxia

Calpain-Inhibitors Protect Frataxin-Deficient Dorsal Root Ganglia Neurons from Loss of Mitochondrial Na+/Ca2+ Exchanger, NCLX, and Apoptosis

(2020) Elena Britti et al.   NEUROCHEMICAL RESEARCH

Cofilin dysregulation alters actin turnover in frataxin-deficient neurons

(2020) Diana C. Muñoz-Lasso et al.   Scientific Reports

Frataxin-deficient cardiomyocytes present an altered thiol-redox state which targets actin and pyruvate dehydrogenase

(2020) Rosa Purroy et al.   Redox Biology

Phosphodiesterase Inhibitors Revert Axonal Dystrophy in Friedreich’s Ataxia Mouse Model

(2019) Belén Mollá et al.   Neurotherapeutics

Regulation of Mitochondrial Biogenesis as a Way for Active Longevity: Interaction Between the Nrf2 and PGC-1α Signaling Pathways

(2019) Artem P. Gureev et al.   Frontiers in Genetics

New developments in pharmacotherapy for Friedreich ataxia

(2019) Alexandra Clay et al.   EXPERT OPINION ON PHARMACOTHERAPY

Mitochondrial pore opening and loss of Ca 2+ exchanger NCLX levels occur after frataxin depletion

(2018) R. Purroy et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

GRP75 overexpression rescues frataxin deficiency and mitochondrial phenotypes in Friedreich Ataxia cellular models

(2018) Yi Na Dong et al.   HUMAN MOLECULAR GENETICS

Calcium Deregulation: Novel Insights to Understand Friedreich’s Ataxia Pathophysiology

(2018) Rosella Abeti et al.   Frontiers in Cellular Neuroscience

Frataxin deficiency impairs mitochondrial biogenesis in cells, mice and humans

(2017) Mittal J. Jasoliya et al.   HUMAN MOLECULAR GENETICS

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

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

Peroxisome Proliferator-Activated Receptors and the Heart: Lessons from the Past and Future Directions

(2015) Wang-Soo Lee et al.   PPAR Research

15d-Prostaglandin J2 Protects Cortical Neurons Against Oxygen–Glucose Deprivation/Reoxygenation Injury: Involvement of Inhibiting Autophagy Through Upregulation of Bcl-2

(2014) Haidong Qin et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Frataxin deficiency in neonatal rat ventricular myocytes targets mitochondria and lipid metabolism

(2014) Èlia Obis et al.   FREE RADICAL BIOLOGY AND MEDICINE

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

Apoptotic cell death and altered calcium homeostasis caused by frataxin depletion in dorsal root ganglia neurons can be prevented by BH4 domain of Bcl-xL protein

(2013) S. Mincheva-Tasheva et al.   HUMAN MOLECULAR GENETICS

Iron–sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia

(2012) Rachael A. Vaubel et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Changes in mitochondrial glutathione levels and protein thiol oxidation in ∆yfh1 yeast cells and the lymphoblasts of patients with Friedreich's ataxia

(2011) A.L. Bulteau et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mortality in Friedreich Ataxia

(2011) Amy Y. Tsou et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Friedreich's ataxia: Pathology, pathogenesis, and molecular genetics

(2011) Arnulf H. Koeppen   JOURNAL OF THE NEUROLOGICAL SCIENCES

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

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

PGC-1alpha Down-Regulation Affects the Antioxidant Response in Friedreich's Ataxia

(2010) Daniele Marmolino et al.   PLoS One

The dorsal root ganglion in Friedreich’s ataxia

(2009) Arnulf H. Koeppen et al.   ACTA NEUROPATHOLOGICA

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

Rosiglitazone and PPAR-γ overexpression protect mitochondrial membrane potential and prevent apoptosis by upregulating anti-apoptotic Bcl-2 family proteins

(2009) Jui-Sheng Wu et al.   JOURNAL OF CELLULAR PHYSIOLOGY

PPAR-γ Agonist Azelaoyl PAF Increases Frataxin Protein and mRNA Expression. New Implications for the Friedreich’s Ataxia Therapy

(2008) Daniele Marmolino et al.   CEREBELLUM