Overexpression of Drosophila frataxin triggers cell death in an iron-dependent manner

E3 Ligase RNF126 Directly Ubiquitinates Frataxin, Promoting Its Degradation: Identification of a Potential Therapeutic Target for Friedreich Ataxia

(2017) Monica Benini et al.   Cell Reports

Auxilin Underlies Progressive Locomotor Deficits and Dopaminergic Neuron Loss in a Drosophila Model of Parkinson’s Disease

(2017) Li Song et al.   Cell Reports

Drosophilatools and assays for the study of human diseases

(2016) Berrak Ugur et al.   Disease Models & Mechanisms

Development of an AAV9 coding for a 3XFLAG-TALEfrat#8-VP64 able to increase in vivo the human frataxin in YG8R mice

(2016) P Chapdelaine et al.   GENE THERAPY

Metabolic regulation of mitochondrial dynamics

(2016) Prashant Mishra et al.   JOURNAL OF CELL BIOLOGY

Metal Homeostasis Regulators Suppress FRDA Phenotypes in a Drosophila Model of the Disease

(2016) Sirena Soriano et al.   PLoS One

Activating frataxin expression by repeat-targeted nucleic acids

(2016) Liande Li et al.   Nature Communications

Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich’s ataxia

(2016) Joseph F. Nabhan et al.   Scientific Reports

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

Translating HDAC inhibitors in Friedreich’s ataxia

(2016) Elisabetta Soragni et al.   Expert Opinion on Orphan Drugs

Activation of mitochondrial energy metabolism protects against cardiac failure

(2016) Tim J. Schulz et al.   Aging-US

Inhibition of alpha-synuclein aggregation by multifunctional dopamine agonists assessed by a novel in vitro assay and an in vivo Drosophila synucleinopathy model

(2016) Deepthi Yedlapudi et al.   Scientific Reports

Frataxin Accelerates [2Fe-2S] Cluster Formation on the Human Fe–S Assembly Complex

(2015) Nicholas G. Fox et al.   BIOCHEMISTRY

Iron-Starvation-Induced Mitophagy Mediates Lifespan Extension upon Mitochondrial Stress in C. elegans

(2015) Alfonso Schiavi et al.   CURRENT BIOLOGY

Mitoferrin modulates iron toxicity in a Drosophila model of Friedreich׳s ataxia

(2015) Juan A. Navarro et al.   FREE RADICAL BIOLOGY AND MEDICINE

Frataxin inactivation leads to steroid deficiency in flies and human ovarian cells

(2015) Amandine Palandri et al.   HUMAN MOLECULAR GENETICS

Src inhibitors modulate frataxin protein levels

(2015) Fabio Cherubini et al.   HUMAN MOLECULAR GENETICS

Anti-Aβ single-chain variable fragment antibodies exert synergistic neuroprotective activities inDrosophilamodels of Alzheimer's disease

(2015) Pedro Fernandez-Funez et al.   HUMAN MOLECULAR GENETICS

Mitochondrial iron supply is required for the developmental pulse of ecdysone biosynthesis that initiates metamorphosis in Drosophila melanogaster

(2015) Jose V. Llorens et al.   JOURNAL OF BIOLOGICAL INORGANIC CHEMISTRY

Highly specific ubiquitin-competing molecules effectively promote frataxin accumulation and partially rescue the aconitase defect in Friedreich ataxia cells

(2015) Alessandra Rufini et al.   NEUROBIOLOGY OF DISEASE

A Yeast/DrosophilaScreen to Identify New Compounds Overcoming Frataxin Deficiency

(2015) Alexandra Seguin et al.   Oxidative Medicine and Cellular Longevity

MiRNA-145 Regulates the Development of Congenital Heart Disease Through Targeting FXN

(2015) Lei Wang et al.   PEDIATRIC CARDIOLOGY

Mechanism of Action of 2-Aminobenzamide HDAC Inhibitors in Reversing Gene Silencing in Friedreich’s Ataxia

(2015) Elisabetta Soragni et al.   Frontiers in Neurology

Epigenetic therapy for Friedreich ataxia

(2014) Elisabetta Soragni et al.   ANNALS OF NEUROLOGY

Human Frataxin Activates Fe–S Cluster Biosynthesis by Facilitating Sulfur Transfer Chemistry

(2014) Jennifer Bridwell-Rabb et al.   BIOCHEMISTRY

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

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

Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia

(2014) Sunil Sahdeo et al.   HUMAN MOLECULAR GENETICS

Analysis of dopaminergic neuronal dysfunction in genetic and toxin-induced models of Parkinson's disease inDrosophila

(2014) Juan A. Navarro et al.   JOURNAL OF NEUROCHEMISTRY

Vacuolar protein sorting 35 (Vps35) rescues locomotor deficits and shortened lifespan in Drosophila expressing a Parkinson’s disease mutant of Leucine-rich repeat kinase 2 (LRRK2)

(2014) Radek Linhart et al.   Molecular Neurodegeneration

Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia

(2014) Morgane Perdomini et al.   NATURE MEDICINE

Muscle Mitohormesis Promotes Longevity via Systemic Repression of Insulin Signaling

(2013) Edward Owusu-Ansah et al.   CELL

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

(2013) Sirena Soriano et al.   GENE

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

(2013) Hervé Tricoire et al.   HUMAN MOLECULAR GENETICS

Friedreich Ataxia: Neuropathology Revised

(2013) Arnulf H. Koeppen et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Probing Mechanisms That Underlie Human Neurodegenerative Diseases inDrosophila

(2012) M. Jaiswal et al.   Annual Review of Genetics

Oxidative stress induces mitochondrial fragmentation in frataxin-deficient cells

(2012) Sophie Lefevre et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Tat-Frataxin protects dopaminergic neuronal cells against MPTP-induced toxicity in a mouse model of Parkinson's disease

(2012) Mi Jin Kim et al.   BIOCHIMIE

Interferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model

(2012) B. Tomassini et al.   HUMAN MOLECULAR GENETICS

Fiji: an open-source platform for biological-image analysis

(2012) Johannes Schindelin et al.   NATURE METHODS

Mitochondrial dynamics in neurodegeneration

(2012) Kie Itoh et al.   TRENDS IN CELL BIOLOGY

Extending life span by increasing oxidative stress

(2011) Michael Ristow et al.   FREE RADICAL BIOLOGY AND MEDICINE

Preventing the ubiquitin–proteasome-dependent degradation of frataxin, the protein defective in Friedreich's ataxia

(2011) Alessandra Rufini et al.   HUMAN MOLECULAR GENETICS

Overexpression of Human and Fly Frataxins in Drosophila Provokes Deleterious Effects at Biochemical, Physiological and Developmental Levels

(2011) Juan A. Navarro et al.   PLoS One

How increased oxidative stress promotes longevity and metabolic health: The concept of mitochondrial hormesis (mitohormesis)

(2010) Michael Ristow et al.   EXPERIMENTAL GERONTOLOGY

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

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

Overexpression ofDrosophila mitoferrinin l(2)mbn cells results in dysregulation of Fer1HCH expression

(2009) Christoph Metzendorf et al.   BIOCHEMICAL JOURNAL

The Drosophila PGC-1 homologue Spargel coordinates mitochondrial activity to insulin signalling

(2009) Stefanie K Tiefenböck et al.   EMBO JOURNAL

Overexpression of the yeast frataxin homolog (Yfh1): Contrasting effects on iron–sulfur cluster assembly, heme synthesis and resistance to oxidative stress

(2009) Alexandra Seguin et al.   MITOCHONDRION

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

Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant

(2009) M. L.-H. Huang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The PINK1–Parkin pathway is involved in the regulation of mitochondrial remodeling process

(2008) Jeehye Park et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

DrosophilaFrataxin: An Iron Chaperone during Cellular Fe−S Cluster Bioassembly†

(2008) Kalyan C. Kondapalli et al.   BIOCHEMISTRY

Recombinant Human Erythropoietin Increases Frataxin Protein Expression Without Increasing mRNA Expression

(2008) Fabio Acquaviva et al.   CEREBELLUM

Keap1/Nrf2 Signaling Regulates Oxidative Stress Tolerance and Lifespan in Drosophila

(2008) Gerasimos P. Sykiotis et al.   DEVELOPMENTAL CELL

Overexpression of frataxin in the mitochondria increases resistance to oxidative stress and extends lifespan inDrosophila

(2008) Alexander P. Runko et al.   FEBS LETTERS

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

The Parkinson's disease genes pink1 and parkin promote mitochondrial fission and/or inhibit fusion in Drosophila

(2008) H. Deng et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA