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。A review on iron chelators as potential therapeutic agents for the treatment of Alzheimer's and Parkinson's diseases
Yash Pal Singh et al.
MOLECULAR DIVERSITY (2019)
The role of oxidative stress in Friedreich's ataxia
Federica Lupoli et al.
FEBS LETTERS (2018)
Impact of Drosophila Models in the Study and Treatment of Friedreich's Ataxia
Veronique Monnier et al.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2018)
Interactions of iron-bound frataxin with ISCU and ferredoxin on the cysteine desulfurase complex leading to Fe-S cluster assembly
Kai Cai et al.
JOURNAL OF INORGANIC BIOCHEMISTRY (2018)
Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia
Francoise Piguet et al.
MOLECULAR THERAPY (2018)
Cerebral abnormalities in Friedreich ataxia: A review
Louisa P. Selvadurai et al.
NEUROSCIENCE AND BIOBEHAVIORAL REVIEWS (2018)
Drosophila melanogaster Models of Friedreich's Ataxia
P. Calap-Quintana et al.
BIOMED RESEARCH INTERNATIONAL (2018)
Mitofusin-Dependent ER Stress Triggers Glial Dysfunction and Nervous System Degeneration in a Drosophila Model of Friedreich's Ataxia
Oliver Edenharter et al.
FRONTIERS IN MOLECULAR NEUROSCIENCE (2018)
Characterization of a new N-terminally acetylated extra-mitochondrial isoform of frataxin in human erythrocytes
Lili Guo et al.
SCIENTIFIC REPORTS (2018)
Iron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon?
David Alsina et al.
PHARMACEUTICALS (2018)
Pharmacological therapeutics in Friedreich ataxia: the present state
Cassandra Strawser et al.
EXPERT REVIEW OF NEUROTHERAPEUTICS (2017)
Nrf2-Inducers Counteract Neurodegeneration in Frataxin-Silenced Motor Neurons: Disclosing New Therapeutic Targets for Friedreich's Ataxia
Sara Petrillo et al.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2017)
Iron-induced oligomerization of human FXN81-210 and bacterial CyaY frataxin and the effect of iron chelators
Eva-Christina Ahlgren et al.
PLOS ONE (2017)
Transplantation of wild-type mouse hematopoietic stem and progenitor cells ameliorates deficits in a mouse model of Friedreich's ataxia
Celine J. Rocca et al.
SCIENCE TRANSLATIONAL MEDICINE (2017)
Inducible and reversible phenotypes in a novel mouse model of Friedreich's Ataxia
Vijayendran Chandran et al.
ELIFE (2017)
Pharmacological therapeutics in Friedreich ataxia: the present state
Cassandra Strawser et al.
EXPERT REVIEW OF NEUROTHERAPEUTICS (2017)
Reversible Axonal Dystrophy by Calcium Modulation in Frataxin-Deficient Sensory Neurons of YG8R Mice
Belen Molla et al.
FRONTIERS IN MOLECULAR NEUROSCIENCE (2017)
Development of an iron-selective antioxidant probe with protective effects on neuronal function
Olimpo Garcia-Beltran et al.
PLOS ONE (2017)
Abundance and Significance of Iron, Zinc, Copper, and Calcium in the Hearts of Patients With Friedreich Ataxia
Pamela C. Kruger et al.
AMERICAN JOURNAL OF CARDIOLOGY (2016)
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)
A reduction in Drp1-mediated fission compromises mitochondrial health in autosomal recessive spastic ataxia of Charlevoix Saguenay
Teisha Y. Bradshaw et al.
HUMAN MOLECULAR GENETICS (2016)
Frataxin silencing alters microtubule stability in motor neurons: implications for Friedreich's ataxia
Emanuela Piermarini et al.
HUMAN MOLECULAR GENETICS (2016)
Efficient attenuation of Friedreich's ataxia (FRDA) cardiomyopathy by modulation of iron homeostasis-human induced pluripotent stem cell (hiPSC) as a drug screening platform for FRDA
Yee-Ki Lee et al.
INTERNATIONAL JOURNAL OF CARDIOLOGY (2016)
Clinical Experience With Deferiprone Treatment for Friedreich Ataxia
Sandra Elincx-Benizri et al.
JOURNAL OF CHILD NEUROLOGY (2016)
Role of iron in neurodegenerative diseases
Kai Li et al.
JOURNAL OF NEURAL TRANSMISSION (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)
Gene Transfer of Brain-derived Neurotrophic Factor (BDNF) Prevents Neurodegeneration Triggered by FXN Deficiency
Yurika Katsu-Jimenez et al.
MOLECULAR THERAPY (2016)
Metal Homeostasis Regulators Suppress FRDA Phenotypes in a Drosophila Model of the Disease
Sirena Soriano et al.
PLOS ONE (2016)
The mitochondrial acyl carrier protein (ACP) coordinates mitochondrial fatty acid synthesis with iron sulfur cluster biogenesis
Jonathan G. Van Vranken et al.
ELIFE (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)
Translating HDAC inhibitors in Friedreich's ataxia
Elisabetta Soragni et al.
EXPERT OPINION ON ORPHAN DRUGS (2016)
Dorsal root ganglia in Friedreich ataxia: satellite cell proliferation and inflammation
Arnulf H. Koeppen et al.
ACTA NEUROPATHOLOGICA COMMUNICATIONS (2016)
The novel mitochondrial iron chelator 5-((methylamino)methyl)-8-hydroxyquinoline protects against mitochondrial-induced oxidative damage and neuronal death
Natalia P. Mena et al.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2015)
Frataxin Accelerates [2Fe-2S] Cluster Formation on the Human Fe-S Assembly Complex
Nicholas G. Fox et al.
BIOCHEMISTRY (2015)
Cellular iron uptake, trafficking and metabolism: Key molecules and mechanisms and their roles in disease
D. J. R. Lane 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)
The role of mitochondria and the CIA machinery in the maturation of cytosolic and nuclear iron-sulfur proteins
Roland Lill et al.
EUROPEAN JOURNAL OF CELL BIOLOGY (2015)
Mitoferrin modulates iron toxicity in a Drosophila model of Friedreich's ataxia
Juan A. Navarro et al.
FREE RADICAL BIOLOGY AND MEDICINE (2015)
The deferiprone and deferasirox combination is efficacious in iron overloaded patients with -thalassemia major: A prospective, single center, open-label study
Sidharth Totadri et al.
PEDIATRIC BLOOD & CANCER (2015)
The Pathogenesis of Cardiomyopathy in Friedreich Ataxia
Arnulf H. Koeppen et al.
PLOS ONE (2015)
Frataxin Is Localized to Both the Chloroplast and Mitochondrion and Is Involved in Chloroplast Fe-S Protein Function in Arabidopsis
Valeria R. Turowski et al.
PLOS ONE (2015)
Unveiling a common mechanism of apoptosis in β-cells and neurons in Friedreich's ataxia
Mariana Igoillo-Esteve et al.
HUMAN MOLECULAR GENETICS (2015)
Deferiprone in Friedreich Ataxia: A 6-Month Randomized Controlled Trial
Massimo Pandolfo et al.
ANNALS OF NEUROLOGY (2014)
Mammalian Fe-S cluster biogenesis and its implication in disease
Lena K. Beilschmidt et al.
BIOCHIMIE (2014)
Dentate nuclei T2 relaxometry is a reliable neuroimaging marker in Friedreich's ataxia
C. Bonilha da Silva et al.
EUROPEAN JOURNAL OF NEUROLOGY (2014)
Frataxin deficiency in neonatal rat ventricular myocytes targets mitochondria and lipid metabolism
Elia Obis et al.
FREE RADICAL BIOLOGY AND MEDICINE (2014)
Heme and erythropoieis: more than a structural role
Deborah Chiabrando et al.
HAEMATOLOGICA (2014)
Methylene blue rescues heart defects in a Drosophila model of Friedreich's ataxia
Herve Tricoire et al.
HUMAN MOLECULAR GENETICS (2014)
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
Stefka Mincheva-Tasheva et al.
HUMAN MOLECULAR GENETICS (2014)
Frataxin Silencing Inactivates Mitochondrial Complex I in NSC34 Motoneuronal Cells and Alters Glutathione Homeostasis
Barbara Carletti et al.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2014)
Inhibition of Neuronal Cell Mitochondrial Complex I with Rotenone Increases Lipid β-Oxidation, Supporting Acetyl-Coenzyme A Levels
Andrew J. Worth et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2014)
Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia
Morgane Perdomini et al.
NATURE MEDICINE (2014)
Mitochondrial dysfunction induced by frataxin deficiency is associated with cellular senescence and abnormal calcium metabolism
Arantxa Bolinches-Amoros et al.
FRONTIERS IN CELLULAR NEUROSCIENCE (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)
Cerebellar pathology in Friedreich's ataxia: Atrophied dentate nuclei with normal iron content
K. Solbach et al.
NEUROIMAGE-CLINICAL (2014)
Molecular and Functional Alterations in a Mouse Cardiac Model of Friedreich Ataxia Activation of the Integrated Stress Response, eIF2α Phosphorylation, and the Induction of Downstream Targets
Michael Li-Hsuan Huang et al.
AMERICAN JOURNAL OF PATHOLOGY (2013)
Frataxin Deficiency Leads to Defects in Expression of Antioxidants and Nrf2 Expression in Dorsal Root Ganglia of the Friedreich's Ataxia YG8R Mouse Model
Yuxi Shan et al.
ANTIOXIDANTS & REDOX SIGNALING (2013)
His86 from the N-Terminus of Frataxin Coordinates Iron and Is Required for Fe-S Cluster Synthesis
Leslie E. Gentry et al.
BIOCHEMISTRY (2013)
Autophagy induction extends lifespan and reduces lipid content in response to frataxin silencing in C. elegans
Alfonso Schiavi et al.
EXPERIMENTAL GERONTOLOGY (2013)
Deferiprone and idebenone rescue frataxin depletion phenotypes in a Drosophila model of Friedreich's ataxia
Sirena Soriano et al.
GENE (2013)
Frataxin Deficiency Leads to Reduced Expression and Impaired Translocation of NF-E2-Related Factor (Nrf2) in Cultured Motor Neurons
Valentina D'Oria et al.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2013)
Frataxin Directly Stimulates Mitochondrial Cysteine Desulfurase by Exposing Substrate-binding Sites, and a Mutant Fe-S Cluster Scaffold Protein with Frataxin-bypassing Ability Acts Similarly
Alok Pandey et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2013)
Nikolaus Friedreich and degenerative atrophy of the dorsal columns of the spinal cord
Arnulf H. Koeppen
JOURNAL OF NEUROCHEMISTRY (2013)
Mitochondrial pathophysiology in Friedreich's ataxia
Pilar Gonzalez-Cabo et al.
JOURNAL OF NEUROCHEMISTRY (2013)
Prevalence gradients of Friedreich's Ataxia and R1b haplotype in Europe co-localize, suggesting a common Palaeolithic origin in the Franco-Cantabrian ice age refuge
Pierre Vankan
JOURNAL OF NEUROCHEMISTRY (2013)
Cardiomyopathy of Friedreich Ataxia
Frank Weidemann et al.
JOURNAL OF NEUROCHEMISTRY (2013)
Clinical features of Friedreich's ataxia: classical and atypical phenotypes
Michael H. Parkinson et al.
JOURNAL OF NEUROCHEMISTRY (2013)
Friedreich Ataxia: Neuropathology Revised
Arnulf H. Koeppen et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2013)
Mammalian Frataxin Controls Sulfur Production and Iron Entry during de Novo Fe4S4 Cluster Assembly
Florent Colin et al.
JOURNAL OF THE AMERICAN CHEMICAL SOCIETY (2013)
Erythroid Heme Biosynthesis and Its Disorders
Harry A. Dailey et al.
COLD SPRING HARBOR PERSPECTIVES IN MEDICINE (2013)
Regional siderosis: a new challenge for iron chelation therapy
Zvi Ioav Cabantchik et al.
FRONTIERS IN PHARMACOLOGY (2013)
Structural imaging differences and longitudinal changes in primary lateral sclerosis and amyotrophic lateral sclerosis
Justin Y. Kwan et al.
NEUROIMAGE-CLINICAL (2013)
Friedreich ataxia: metal dysmetabolism in dorsal root ganglia
Arnulf H. Koeppen et al.
ACTA NEUROPATHOLOGICA COMMUNICATIONS (2013)
Relation of Cytosolic Iron Excess to Cardiomyopathy of Friedreich's Ataxia
R. Liane Ramirez et al.
AMERICAN JOURNAL OF CARDIOLOGY (2012)
Effector Role Reversal during Evolution: The Case of Frataxin in Fe-S Cluster Biosynthesis
Jennifer Bridwell-Rabb et al.
BIOCHEMISTRY (2012)
Ferroptosis: An Iron-Dependent Form of Nonapoptotic Cell Death
Scott J. Dixon et al.
CELL (2012)
Sphingolipid Signaling Mediates Iron Toxicity
Yueh-Jung Lee et al.
CELL METABOLISM (2012)
Friedreich's Ataxia Causes Redistribution of Iron, Copper, and Zinc in the Dentate Nucleus
Arnulf H. Koeppen et al.
CEREBELLUM (2012)
Heme metabolism and erythropoiesis
Jacky Chung et al.
CURRENT OPINION IN HEMATOLOGY (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)
A Role for Iron-Sulfur Clusters in the Regulation of Transcription Factor Yap5-dependent High Iron Transcriptional Responses in Yeast
Liangtao Li et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2012)
Cardiomyopathy in Friedreich Ataxia: Clinical Findings and Research
R. Mark Payne et al.
JOURNAL OF CHILD NEUROLOGY (2012)
Elevated brain iron is independent from atrophy in Huntington's Disease
Eve M. Dumas et al.
NEUROIMAGE (2012)
Identification of nonferritin mitochondrial iron deposits in a mouse model of Friedreich ataxia
Megan Whitnall et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2012)
Is Friedreich ataxia an epigenetic disorder?
Daman Kumari et al.
CLINICAL EPIGENETICS (2012)
Mitochondrial Mayhem: The Mitochondrion as a Modulator of Iron Metabolism and Its Role in Disease
Michael Li-Hsuan Huang et al.
ANTIOXIDANTS & REDOX SIGNALING (2011)
Structure-Function Analysis of Friedreich's Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe-S Assembly Complex
Jennifer Bridwell-Rabb et al.
BIOCHEMISTRY (2011)
Friedreich's ataxia: Past, present and future
Daniele Marmolino
BRAIN RESEARCH REVIEWS (2011)
Impaired myocardial perfusion reserve and fibrosis in Friedreich ataxia: a mitochondrial cardiomyopathy with metabolic syndrome
Subha V. Raman et al.
EUROPEAN HEART JOURNAL (2011)
Is Prevalence of Retinopathy Related to the Age of Onset of Diabetes? Sankara Nethralaya Diabetic Retinopathy Epidemiology and Molecular Genetic Report No. 5
Rajiv Raman et al.
OPHTHALMIC RESEARCH (2011)
Mammalian Frataxin: An Essential Function for Cellular Viability through an Interaction with a Preformed ISCU/NFS1/ISD11 Iron-Sulfur Assembly Complex
Stephane Schmucker et al.
PLOS ONE (2011)
Congenital Sideroblastic Anemias: Iron and Heme Lost in Mitochondrial Translation
Mark D. Fleming
HEMATOLOGY-AMERICAN SOCIETY OF HEMATOLOGY EDUCATION PROGRAM (2011)
Human Frataxin Is an Allosteric Switch That Activates the Fe-S Cluster Biosynthetic Complex
Chi-Lin Tsai et al.
BIOCHEMISTRY (2010)
Altered lipid metabolism in a Drosophila model of Friedreich's ataxia
Juan A. Navarro et al.
HUMAN MOLECULAR GENETICS (2010)
Molecular control of the cytosolic aconitase/IRP1 switch by extramitochondrial frataxin
Ivano Condo et al.
HUMAN MOLECULAR GENETICS (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)
Analysis of the Factors Influencing the Cardiac Phenotype in Friedreich's Ataxia
Bheeshma Rajagopalan et al.
MOVEMENT DISORDERS (2010)
Building Fe-S proteins: bacterial strategies
Beatrice Py et al.
NATURE REVIEWS MICROBIOLOGY (2010)
Mitochondrial iron trafficking and the integration of iron metabolism between the mitochondrion and cytosol
Des R. Richardson et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
The dorsal root ganglion in Friedreich's ataxia
Arnulf H. Koeppen et al.
ACTA NEUROPATHOLOGICA (2009)
Nitric oxide accumulation is required to protect against iron-mediated oxidative stress in frataxin-deficient Arabidopsis plants
Mariana Martin et al.
FEBS LETTERS (2009)
Mitochondrial ferritin limits oxidative damage regulating mitochondrial iron availability: hypothesis for a protective role in Friedreich ataxia
Alessandro Campanella et al.
HUMAN MOLECULAR GENETICS (2009)
The pathogenesis of Friedreich ataxia and the structure and function of frataxin
Massimo Pandolfo et al.
JOURNAL OF NEUROLOGY (2009)
Function and biogenesis of iron-sulphur proteins
Roland Lill
NATURE (2009)
Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS
Salvatore Adinolfi et al.
NATURE STRUCTURAL & MOLECULAR BIOLOGY (2009)
Impaired Nuclear Nrf2 Translocation Undermines the Oxidative Stress Response in Friedreich Ataxia
Vincent Paupe et al.
PLOS ONE (2009)
The Longitudinal Course of Cardiomyopathy in Friedreich's Ataxia During Childhood
Alaina Kipps et al.
PEDIATRIC CARDIOLOGY (2009)
Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant
Michael Li-Hsuan Huang et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation
Or Kakhlon et al.
BLOOD (2008)
Mapping iron binding sites on human frataxin: implications for cluster assembly on the ISUFe-S cluster scaffold protein
Jia Huang et al.
JOURNAL OF BIOLOGICAL INORGANIC CHEMISTRY (2008)
The MCK mouse heart model of Friedreich's ataxia: Alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation
Megan Whitnall et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (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)
Redistribution of accumulated cell iron: a modality of chelation with therapeutic implications
Yang-Sung Sohn et al.
BLOOD (2008)
Partial conservation of functions between eukaryotic frataxin and the Escherichia coli frataxin homolog CyaY
Tibor Bedekovics et al.
FEMS YEAST RESEARCH (2007)
The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteins
Arnulf H. Koeppen et al.
ACTA NEUROPATHOLOGICA (2007)
In vivo maturation of human frataxin
Ivano Condo et al.
HUMAN MOLECULAR GENETICS (2007)
N-terminal iron-mediated self-cleavage of human frataxin: regulation of iron binding and complex formation with target proteins
Taejin Yoon et al.
JOURNAL OF BIOLOGICAL INORGANIC CHEMISTRY (2007)
Knockout of frataxin gene causes embryo lethality in Arabidopsis
Valentina Vazzola et al.
FEBS LETTERS (2007)
Causative role of oxidative stress in a Drosophila model of Friedreich ataxia
Jose V. Llorens et al.
FASEB JOURNAL (2007)
Acidic residues of yeast frataxin have an essential role in Fe-S cluster assembly
Francoise Foury et al.
EMBO REPORTS (2007)
Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts
Chunye Lu et al.
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS (2007)
Heme as a magnificent molecule with multiple missions: Heme determines its own fate and governs cellular homeostasis
Kazumichi Furuyama et al.
TOHOKU JOURNAL OF EXPERIMENTAL MEDICINE (2007)
Modern diagnosis and management of the porphyrias
Shigeru Sassa
BRITISH JOURNAL OF HAEMATOLOGY (2006)
Monomeric yeast frataxin is an iron-binding protein
Jeremy D. Cook et al.
BIOCHEMISTRY (2006)
A pool of extramitochondrial frataxin that promotes cell survival
Ivano Condo et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2006)
Iron-sulfur cluster biosynthesis -: Characterization of Escherichia coli CyaY as an iron donor for the assembly of [2Fe-2S] clusters in the scaffold IscU
Gunhild Layer et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2006)
Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity
O Gakh et al.
HUMAN MOLECULAR GENETICS (2006)
Iron and iron-responsive proteins in the cardiomyopathy of Friedreich's ataxia
Susan Michael et al.
CEREBELLUM (2006)
Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells
RA Schoenfeld et al.
HUMAN MOLECULAR GENETICS (2005)
RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila
PR Anderson et al.
HUMAN MOLECULAR GENETICS (2005)
Extra-mitochondrial localisation of frataxin and its association with IscU1 during enterocyte-like differentiation of the human colon adenocarcinoma cell line Caco-2
F Acquaviva et al.
JOURNAL OF CELL SCIENCE (2005)
Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxia
V Calabrese et al.
JOURNAL OF THE NEUROLOGICAL SCIENCES (2005)
Iron trafficking in the mitochondrion: novel pathways revealed by disease
I Napier et al.
BLOOD (2005)
Friedreich's ataxia, no changes in mitochondrial labile iron in human lymphoblasts and fibroblasts - A decrease in antioxidative capacity?
B Sturm et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Friedreich ataxia: the oxidative stress paradox
H Seznec et al.
HUMAN MOLECULAR GENETICS (2005)
Assembly of human frataxin is a mechanism for detoxifying redox-active iron
HA O'Neill et al.
BIOCHEMISTRY (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)
Iron binding and oxidation kinetics in frataxin CyaY of Escherichia coli
F Bou-Abdallah et al.
JOURNAL OF MOLECULAR BIOLOGY (2004)
Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity
AL Bulteau et al.
SCIENCE (2004)
Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis
T Yoon et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2004)
Iron-induced oligomerization of yeast frataxin homologue Yfh1 is dispensable in vivo
K Aloria et al.
EMBO REPORTS (2004)
An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1
J Gerber et al.
EMBO REPORTS (2003)
Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation
S Park et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2003)
Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins
T Yoon et al.
JOURNAL OF THE AMERICAN CHEMICAL SOCIETY (2003)
DNA triplet repeats mediate heterochromatin-protein-1-sensitive variegated gene silencing
A Saveliev et al.
NATURE (2003)
Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1)
E Lesuisse et al.
HUMAN MOLECULAR GENETICS (2003)
The labile iron pool: Characterization, measurement, and participation in cellular processes
O Kakhlon et al.
FREE RADICAL BIOLOGY AND MEDICINE (2002)
The ferroxidase activity of yeast frataxin
S Park et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2002)
A structural approach to understanding the iron-binding properties of phylogenetically different frataxins
S Adinolfi et al.
HUMAN MOLECULAR GENETICS (2002)
Physical evidence that yeast frataxin is an iron storage protein
O Gakh et al.
BIOCHEMISTRY (2002)
Erythroid differentiation and protoporphyrin IX down-regulate frataxin expression in Friend cells: characterization of frataxin expression compared to molecules involved in iron metabolism and hemoglobinization
EM Becker et al.
BLOOD (2002)
Molecular bases of cellular iron toxicity
JW Eaton et al.
FREE RADICAL BIOLOGY AND MEDICINE (2002)
Frataxin expression rescues mitochondrial dysfunctions in FRDA cells
G Tan et al.
HUMAN MOLECULAR GENETICS (2001)
Disabled early recruitment of antioxidant defenses in Friedreich's ataxia
K Chantrel-Groussard et al.
HUMAN MOLECULAR GENETICS (2001)
The 2.0 Å structure of human ferrochelatase, the terminal enzyme of heme biosynthesis
CK Wu et al.
NATURE STRUCTURAL BIOLOGY (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)
Increased levels of plasma malondialdehyde in Friedreich ataxia
M Emond et al.
NEUROLOGY (2000)
Oxidative stress in patients with Friedreich ataxia
JB Schulz et al.
NEUROLOGY (2000)
Human frataxin maintains mitochondrial iron homeostasis in Saccharomyces cerevisiae
P Cavadini et al.
HUMAN MOLECULAR GENETICS (2000)
Crystal structure of human frataxin
S Dhe-Paganon et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2000)
Iron-dependent self assembly of recombinant yeast frataxin: Implications for Friedreich ataxia
J Adamec et al.
AMERICAN JOURNAL OF HUMAN GENETICS (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)
Genotype and phenotype analysis of Friedreich's ataxia compound heterozygous patients
M De Castro et al.
HUMAN GENETICS (2000)
分享论文
