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

Glial Lipid Droplets and ROS Induced by Mitochondrial Defects Promote Neurodegeneration

(2015) Lucy Liu et al.   CELL

Impaired Mitochondrial Energy Production Causes Light-Induced Photoreceptor Degeneration Independent of Oxidative Stress

(2015) Manish Jaiswal et al.   PLOS BIOLOGY

A Drosophila Genetic Resource of Mutants to Study Mechanisms Underlying Human Genetic Diseases

(2014) Shinya Yamamoto et al.   CELL

Large-scale identification of chemically induced mutations inDrosophila melanogaster

(2014) Nele A. Haelterman et al.   GENOME RESEARCH

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

(2014) Barbara Carletti et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Activation of 3-Phosphoinositide-dependent Kinase 1 (PDK1) and Serum- and Glucocorticoid-induced Protein Kinase 1 (SGK1) by Short-chain Sphingolipid C4-ceramide Rescues the Trafficking Defect of ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator (ΔF508-CFTR)

(2014) Hung Caohuy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial fusion but not fission regulates larval growth and synaptic development through steroid hormone production

(2014) Hector Sandoval et al.   eLife

Cerebellar pathology in Friedreich's ataxia: Atrophied dentate nuclei with normal iron content

(2014) K. Solbach et al.   NeuroImage-Clinical

MEF2 Is an In Vivo Immune-Metabolic Switch

(2013) Rebecca I. Clark et al.   CELL

The MEF2 family and the brain: from molecules to memory

(2013) Jean-Bernard Dietrich   CELL AND TISSUE RESEARCH

Methylene blue rescues heart defects in a Drosophila model of Friedreich's ataxia

(2013) Hervé Tricoire et al.   HUMAN MOLECULAR GENETICS

The C8ORF38 homologue Sicily is a cytosolic chaperone for a mitochondrial complex I subunit

(2013) Ke Zhang et al.   JOURNAL OF CELL BIOLOGY

Co-enzyme Q10and idebenone use in Friedreich's ataxia

(2013) Michael H Parkinson et al.   JOURNAL OF NEUROCHEMISTRY

A Global In Vivo Drosophila RNAi Screen Identifies a Key Role of Ceramide Phosphoethanolamine for Glial Ensheathment of Axons

(2013) Aniket Ghosh et al.   PLoS Genetics

Sphingolipid Signaling Mediates Iron Toxicity

(2012) Yueh-Jung Lee et al.   Cell Metabolism

Friedreich's Ataxia Causes Redistribution of Iron, Copper, and Zinc in the Dentate Nucleus

(2012) Arnulf H. Koeppen et al.   CEREBELLUM

In Vivo Analysis of MEF2 Transcription Factors in Synapse Regulation and Neuronal Survival

(2012) M. Waseem Akhtar et al.   PLoS One

Gene Regulatory Networks Controlling Hematopoietic Progenitor Niche Cell Production and Differentiation in the Drosophila Lymph Gland

(2012) Yumiko Tokusumi et al.   PLoS One

Identification of nonferritin mitochondrial iron deposits in a mouse model of Friedreich ataxia

(2012) M. Whitnall et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Yeast Complex I Equivalent NADH Dehydrogenase Rescues pink1 Mutants

(2012) Sven Vilain et al.   PLoS Genetics

Friedreich’s Ataxia Variants I154F and W155R Diminish Frataxin-Based Activation of the Iron–Sulfur Cluster Assembly Complex

(2011) Chi-Lin Tsai et al.   BIOCHEMISTRY

Friedreich's ataxia: the vicious circle hypothesis revisited

(2011) Aurélien Bayot et al.   BMC Medicine

Sphingosine kinases and their metabolites modulate endolysosomal trafficking in photoreceptors

(2011) Ikuko Yonamine et al.   JOURNAL OF CELL BIOLOGY

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

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

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

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

A Phase 3, Double-blind, Placebo-Controlled Trial of Idebenone in Friedreich Ataxia

(2010) David R. Lynch et al.   ARCHIVES OF NEUROLOGY

Evidence that yeast frataxin is not an iron storage protein in vivo

(2010) Alexandra Seguin et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

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

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

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

The Transcription Factor Mef2 Is Required for Normal Circadian Behavior in Drosophila

(2010) F. J. Blanchard et al.   JOURNAL OF NEUROSCIENCE

The dorsal root ganglion in Friedreich’s ataxia

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

Function and biogenesis of iron–sulphur proteins

(2009) Roland Lill   NATURE

Versatile P[acman] BAC libraries for transgenesis studies in Drosophila melanogaster

(2009) Koen J T Venken et al.   NATURE METHODS

Accumulation of Rhodopsin in Late Endosomes Triggers Photoreceptor Cell Degeneration

(2009) Yashodhan Chinchore et al.   PLoS Genetics

Distinct mitochondrial retrograde signals control the G1-S cell cycle checkpoint

(2008) Edward Owusu-Ansah et al.   NATURE GENETICS

Principles of bioactive lipid signalling: lessons from sphingolipids

(2008) Yusuf A. Hannun et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

mef2 activity levels differentially affect gene expression during Drosophila muscle development

(2008) S. J. Elgar et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

N-acetylcysteine amide (AD4) attenuates oxidative stress in beta-thalassemia blood cells

(2007) Johnny Amer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS