相关参考文献
注意:仅列出部分参考文献,下载原文获取全部文献信息。Iron Sulfur and Molybdenum Cofactor Enzymes Regulate the Drosophila Life Cycle by Controlling Cell Metabolism
Zvonimir Marelja et al.
FRONTIERS IN PHYSIOLOGY (2018)
Overexpression of Drosophila frataxin triggers cell death in an iron-dependent manner
Oliver Edenharter et al.
JOURNAL OF NEUROGENETICS (2017)
In vitro characterization of a novel Isu homologue from Drosophila melanogaster for de novo FeS-cluster formation
Stephen P. Dzul et al.
METALLOMICS (2017)
Compound Heterozygous FXN Mutations and Clinical Outcome in Friedreich Ataxia
Charles A. Galea et al.
ANNALS OF NEUROLOGY (2016)
Frataxin and the molecular mechanism of mitochondrial iron-loading in Friedreich's ataxia
Shannon Chiang et al.
CLINICAL SCIENCE (2016)
Oxidative stress and altered lipid metabolism in Friedreich ataxia
Jordi Tamarit et al.
FREE RADICAL BIOLOGY AND MEDICINE (2016)
Clinical Experience With Deferiprone Treatment for Friedreich Ataxia
Sandra Elincx-Benizri et al.
JOURNAL OF CHILD NEUROLOGY (2016)
Tissue atrophy and elevated iron concentration in the extrapyramidal motor system in Friedreich ataxia: the IMAGE-FRDA study
Ian H. Harding et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2016)
MEGA7: Molecular Evolutionary Genetics Analysis Version 7.0 for Bigger Datasets
Sudhir Kumar et al.
MOLECULAR BIOLOGY AND EVOLUTION (2016)
Metal Homeostasis Regulators Suppress FRDA Phenotypes in a Drosophila Model of the Disease
Sirena Soriano et al.
PLOS ONE (2016)
Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdkl/Mef2 activation, leading to neurodegeneration
Kuchuan Chen et al.
ELIFE (2016)
Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammals
Kuchuan Chen et al.
ELIFE (2016)
Iron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery
Nunziata Maio et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2015)
Iron Regulatory Protein 1 Sustains Mitochondrial Iron Loading and Function in Frataxin Deficiency
Alain Martelli et al.
CELL METABOLISM (2015)
Mitoferrin modulates iron toxicity in a Drosophila model of Friedreich's ataxia
Juan A. Navarro et al.
FREE RADICAL BIOLOGY AND MEDICINE (2015)
Frataxin inactivation leads to steroid deficiency in flies and human ovarian cells
Amandine Palandri et al.
HUMAN MOLECULAR GENETICS (2015)
The Phyre2 web portal for protein modeling, prediction and analysis
Lawrence A. Kelley et al.
NATURE PROTOCOLS (2015)
A Yeast/Drosophila Screen to Identify New Compounds Overcoming Frataxin Deficiency
Alexandra Seguin et al.
OXIDATIVE MEDICINE AND CELLULAR LONGEVITY (2015)
Targeting lipid peroxidation and mitochondrial imbalance in Friedreich's ataxia
Rosella Abeti et al.
PHARMACOLOGICAL RESEARCH (2015)
TORC1 Inhibition by Rapamycin Promotes Antioxidant Defences in a Drosophila Model of Friedreich's Ataxia
Pablo Calap-Quintana et al.
PLOS ONE (2015)
The Pathogenesis of Cardiomyopathy in Friedreich Ataxia
Arnulf H. Koeppen et al.
PLOS ONE (2015)
Stable isotopes and LC-MS for monitoring metabolic disturbances in Friedreich's ataxia platelets
Andrew J. Worth et al.
BIOANALYSIS (2015)
Mammalian frataxin directly enhances sulfur transfer of NFS1 persulfide to both ISCU and free thiols
Auberie Parent et al.
NATURE COMMUNICATIONS (2015)
Triple therapy with deferiprone, idebenone and riboflavin in Friedreich's ataxia - open-label trial
J. Arpa et al.
ACTA NEUROLOGICA SCANDINAVICA (2014)
Deferiprone in Friedreich Ataxia: A 6-Month Randomized Controlled Trial
Massimo Pandolfo et al.
ANNALS OF NEUROLOGY (2014)
Three conserved histidine residues contribute to mitochondrial iron transport through mitoferrins
Xavier Brazzolotto et al.
BIOCHEMICAL JOURNAL (2014)
Fixing frataxin: 'ironing out' the metabolic defect in Friedreich's ataxia
A. Anzovino et al.
BRITISH JOURNAL OF PHARMACOLOGY (2014)
Methylene blue rescues heart defects in a Drosophila model of Friedreich's ataxia
Herve Tricoire et al.
HUMAN MOLECULAR GENETICS (2014)
The metal transporter ZIP13 supplies iron into the secretory pathway in Drosophila melanogaster
Guiran Xiao et al.
ELIFE (2014)
Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation
Alain Martelli et al.
FRONTIERS IN PHARMACOLOGY (2014)
Therapeutic strategies in Friedreich's Ataxia
Timothy E. Richanison et al.
BRAIN RESEARCH (2013)
Deferiprone and idebenone rescue frataxin depletion phenotypes in a Drosophila model of Friedreich's ataxia
Sirena Soriano et al.
GENE (2013)
Mitochondrial pathophysiology in Friedreich's ataxia
Pilar Gonzalez-Cabo et al.
JOURNAL OF NEUROCHEMISTRY (2013)
Frataxin: a protein in search for a function
Annalisa Pastore et al.
JOURNAL OF NEUROCHEMISTRY (2013)
Animal and cellular models of Friedreich ataxia
Morgane Perdomini et al.
JOURNAL OF NEUROCHEMISTRY (2013)
Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia
Rachael A. Vaubel et al.
MOLECULAR AND CELLULAR NEUROSCIENCE (2013)
Friedreich's Ataxia, Frataxin, PIP5K1B: Echo of a Distant Fracas
Aurelien Bayot et al.
OXIDATIVE MEDICINE AND CELLULAR LONGEVITY (2013)
Neurodegeneration in Friedreich's Ataxia: From Defective Frataxin to Oxidative Stress
Claudio M. Gomes et al.
OXIDATIVE MEDICINE AND CELLULAR LONGEVITY (2013)
Relation of Cytosolic Iron Excess to Cardiomyopathy of Friedreich's Ataxia
R. Liane Ramirez et al.
AMERICAN JOURNAL OF CARDIOLOGY (2012)
Friedreich's Ataxia Causes Redistribution of Iron, Copper, and Zinc in the Dentate Nucleus
Arnulf H. Koeppen et al.
CEREBELLUM (2012)
Clinical data and characterization of the liver conditional mouse model exclude neoplasia as a non-neurological manifestation associated with Friedreich's ataxia
Alain Martelli et al.
DISEASE MODELS & MECHANISMS (2012)
Exploring frataxin function
Maria V. Busi et al.
IUBMB LIFE (2012)
ZIP8 Is an Iron and Zinc Transporter Whose Cell-surface Expression Is Up-regulated by Cellular Iron Loading
Chia-Yu Wang et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2012)
Therapeutic Developments in Friedreich Ataxia
Robert B. Wilson
JOURNAL OF CHILD NEUROLOGY (2012)
Friedreich's Ataxia Variants I154F and W155R Diminish Frataxin-Based Activation of the Iron-Sulfur Cluster Assembly Complex
Chi-Lin Tsai et al.
BIOCHEMISTRY (2011)
Toxicity of Alzheimer's disease-associated Aβ peptide is ameliorated in a Drosophila model by tight control of zinc and copper availability
Haiqing Hua et al.
BIOLOGICAL CHEMISTRY (2011)
Friedreich's ataxia: Past, present and future
Daniele Marmolino
BRAIN RESEARCH REVIEWS (2011)
Combined Therapy with Idebenone and Deferiprone in Patients with Friedreich's Ataxia
Daniel Velasco-Sanchez et al.
CEREBELLUM (2011)
The parkin Mutant Phenotype in the Fly Is Largely Rescued by Metal-Responsive Transcription Factor (MTF-1)
Nidhi Saini et al.
MOLECULAR AND CELLULAR BIOLOGY (2011)
Overexpression of Human and Fly Frataxins in Drosophila Provokes Deleterious Effects at Biochemical, Physiological and Developmental Levels
Juan A. Navarro et al.
PLOS ONE (2011)
Activation of mitochondrial energy metabolism protects against cardiac failure
Tim J. Schulz et al.
AGING-US (2010)
Friedreich Ataxia: Molecular Mechanisms, Redox Considerations, and Therapeutic Opportunities
Renata Santos et al.
ANTIOXIDANTS & REDOX SIGNALING (2010)
Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question
Jeffrey S. Armstrong et al.
FASEB JOURNAL (2010)
Altered lipid metabolism in a Drosophila model of Friedreich's ataxia
Juan A. Navarro et al.
HUMAN MOLECULAR GENETICS (2010)
Frataxin Depletion in Yeast Triggers Up-regulation of Iron Transport Systems before Affecting Iron-Sulfur Enzyme Activities
Armando Moreno-Cermeno et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2010)
Frataxin and Mitochondrial FeS Cluster Biogenesis
Timothy L. Stemmler et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2010)
Defects in Mitochondrial Axonal Transport and Membrane Potential without Increased Reactive Oxygen Species Production in a Drosophila Model of Friedreich Ataxia
Yujiro Shidara et al.
JOURNAL OF NEUROSCIENCE (2010)
Crystal structure of human copper homeostasis protein CutC reveals a potential copper-binding site
Yingjie Li et al.
JOURNAL OF STRUCTURAL BIOLOGY (2010)
Overexpression of metal-responsive transcription factor (MTF-1) in Drosophila melanogaster ameliorates life-span reductions associated with oxidative stress and metal toxicity
Sepehr Bahadorani et al.
NEUROBIOLOGY OF AGING (2010)
Accounting for Solvent Accessibility and Secondary Structure in Protein Phylogenetics Is Clearly Beneficial
Si Quang Le et al.
SYSTEMATIC BIOLOGY (2010)
The dorsal root ganglion in Friedreich's ataxia
Arnulf H. Koeppen et al.
ACTA NEUROPATHOLOGICA (2009)
The yeast mitochondrial carrier proteins Mrs3p/Mrs4p mediate iron transport across the inner mitochondrial membrane
Elisabeth M. Froschauer et al.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES (2009)
The pathogenesis of Friedreich ataxia and the structure and function of frataxin
Massimo Pandolfo et al.
JOURNAL OF NEUROLOGY (2009)
Drosophila frataxin:: An iron chaperone during cellular Fe-S cluster bioassembly
Kalyan C. Kondapalli et al.
BIOCHEMISTRY (2008)
Overexpression of frataxin in the mitochondria increases resistance to oxidative stress and extends lifespan in Drosophila
Alexander P. Runkoa et al.
FEBS LETTERS (2008)
Major targets of iron-induced protein oxidative damage in frataxin-deficient yeasts are magnesium-binding proteins
Veronica Irazusta et al.
FREE RADICAL BIOLOGY AND MEDICINE (2008)
Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia
Peter R. Anderson et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)
The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteins
Arnulf H. Koeppen et al.
ACTA NEUROPATHOLOGICA (2007)
Selective iron chelation in Friedreich ataxia:: biologic and clinical implications
Nathalie Boddaert et al.
BLOOD (2007)
In vivo maturation of human frataxin
Ivano Condo et al.
HUMAN MOLECULAR GENETICS (2007)
Oxidative stress and protease dysfunction in the yeast model of Friedreich ataxia
Anne-Laure Bulteau et al.
FREE RADICAL BIOLOGY AND MEDICINE (2007)
Causative role of oxidative stress in a Drosophila model of Friedreich ataxia
Jose V. Llorens et al.
FASEB JOURNAL (2007)
Deficiency of Arabidopsis thaliana frataxin alters activity of mitochondrial Fe-S proteins and induces oxidative stress
Maria V. Busi et al.
PLANT JOURNAL (2006)
GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology
Sahar Al-Mahdawi et al.
GENOMICS (2006)
Zip14 (Slc39a14) mediates non-transferrin-bound iron uptake into cells
Juan P. Liuzzi et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2006)
A pool of extramitochondrial frataxin that promotes cell survival
Ivano Condo et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2006)
Frataxin, iron-sulfur clusters, heme, ROS, and aging
Eleonora Napoli et al.
ANTIOXIDANTS & REDOX SIGNALING (2006)
Prooxidative effect of copper-metallothionein in the acute cytotoxicity of hydrogen peroxide in Ehrlich ascites tumour cells
ZE Suntres et al.
TOXICOLOGY (2006)
Induction of oxidative metabolism by mitochondrial frataxin inhibits cancer growth - Otto Warburg revisited
TJ Schulz et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2006)
Iron and iron-responsive proteins in the cardiomyopathy of Friedreich's ataxia
Susan Michael et al.
CEREBELLUM (2006)
RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila
PR Anderson et al.
HUMAN MOLECULAR GENETICS (2005)
Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant
RP Vázquez-Manrique et al.
FASEB JOURNAL (2005)
Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia
D Simon et al.
JOURNAL OF NEUROSCIENCE (2004)
Frataxin overexpressing mice
CJ Miranda et al.
FEBS LETTERS (2004)
Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis
T Yoon et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2004)
Candida albicans lacking the frataxin homologue:: a relevant yeast model for studying the role of frataxin
R Santos et al.
MOLECULAR MICROBIOLOGY (2004)
UCSF chimera - A visualization system for exploratory research and analysis
EF Pettersen et al.
JOURNAL OF COMPUTATIONAL CHEMISTRY (2004)
Components involved in assembly and dislocation of iron-sulfur clusters on the scaffold protein Isu1p
U Mühlenhoff et al.
EMBO JOURNAL (2003)
Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1)
E Lesuisse et al.
HUMAN MOLECULAR GENETICS (2003)
Metallochaperone Atox1 transfers copper to the NH2-terminal domain of the Wilson's disease protein and regulates its catalytic activity
JM Walker et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2002)
Near infrared muscle spectroscopy in patients with Friedreich's ataxia
DR Lynch et al.
MUSCLE & NERVE (2002)
Frataxin promotes antioxidant defense in a thiol-dependent manner resulting in diminished malignant transformation in vitro
SA Shoichet et al.
HUMAN MOLECULAR GENETICS (2002)
P{Switch}, a system for spatial and temporal control of gene expression in Drosophila melanogaster
G Roman et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2001)
A conditional tissue-specific transgene expression system using inducible GAL4
T Osterwalder et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2001)
A systematic analysis of human disease-associated gene sequences in Drosophila melanogaster
LT Reiter et al.
GENOME RESEARCH (2001)
Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits
H Puccio et al.
NATURE GENETICS (2001)
Frataxin activates mitochondrial energy conversion and oxidative phosphorylation
M Ristow et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2000)
dfh is a Drosophila homolog of the Friedreich's ataxia disease gene
J Cañizares et al.
GENE (2000)
Heritable gene silencing in Drosophila using double-stranded RNA
JR Kennerdell et al.
NATURE BIOTECHNOLOGY (2000)
A survey of human disease gene counterparts in the Drosophila genome
ME Fortini et al.
JOURNAL OF CELL BIOLOGY (2000)
Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation
M Cossée et al.
HUMAN MOLECULAR GENETICS (2000)
Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia
JL Bradley et al.
HUMAN MOLECULAR GENETICS (2000)