Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches

CTCF: Master Weaver of the Genome

(2009) Jennifer E. Phillips et al.   CELL

Structure-Specific Recognition of Friedreich's Ataxia (GAA)nRepeats by Benzoquinoquinoxaline Derivatives

(2009) Helen Bergquist et al.   CHEMBIOCHEM

Oligomeric Yeast Frataxin Drives Assembly of Core Machinery for Mitochondrial Iron-Sulfur Cluster Synthesis

(2009) Hongqiao Li et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Function and biogenesis of iron–sulphur proteins

(2009) Roland Lill   NATURE

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

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

Epigenetic Silencing in Friedreich Ataxia Is Associated with Depletion of CTCF (CCCTC-Binding Factor) and Antisense Transcription

(2009) Irene De Biase et al.   PLoS One

DNA triplexes and Friedreich ataxia

(2008) Robert D. Wells   FASEB JOURNAL

The in vivo mitochondrial two-step maturation of human frataxin

(2008) Stéphane Schmucker et al.   HUMAN MOLECULAR GENETICS

Assembly of the Iron-binding Protein Frataxin inSaccharomyces cerevisiaeResponds to Dynamic Changes in Mitochondrial Iron Influx and Stress Level

(2008) Oleksandr Gakh et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Binding of Yeast Frataxin to the Scaffold for Fe-S Cluster Biogenesis, Isu

(2008) Tao Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Urinary isoprostanes in Friedreich ataxia: Lack of correlation with disease features

(2008) Lauren M. Myers et al.   MOVEMENT DISORDERS

HDAC Inhibitors Correct Frataxin Deficiency in a Friedreich Ataxia Mouse Model

(2008) Myriam Rai et al.   PLoS One

Iron–sulfur cluster biogenesis and human disease

(2008) Tracey A. Rouault et al.   TRENDS IN GENETICS

Drug Insight: antioxidant therapy in inherited ataxias

(2008) Massimo Pandolfo   Nature clinical practice. Neurology

Antioxidant use in Friedreich ataxia

(2007) Lauren Myers et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES