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Mitochondrial disease: Gene therapy protects heart in Friedreich's ataxia.
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- Nature Reviews Drug Discovery, 2014, v. 13, n. 6, p. 416, doi. 10.1038/nrd4346
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- Article
Radial diffusivity in the cerebellar peduncles correlates with clinical severity in Friedreich ataxia.
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- Neurological Sciences, 2013, v. 34, n. 8, p. 1459, doi. 10.1007/s10072-013-1402-0
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- Article
Variations of frataxin protein levels in normal individuals.
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- Neurological Sciences, 2011, v. 32, n. 2, p. 327, doi. 10.1007/s10072-010-0326-1
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- Article
Absence of aprataxin gene mutations in a Greek cohort with sporadic early onset ataxia and normal GAA triplets in frataxin gene.
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- Neurological Sciences, 2010, v. 31, n. 3, p. 393, doi. 10.1007/s10072-009-0201-0
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- Article
Halogens engineering-based design of agonists for boosting expression of frataxin protein in Friedreich's ataxia.
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- European Review for Medical & Pharmacological Sciences, 2023, v. 27, n. 15, p. 6972
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- Article
DNA methylation in Friedreich ataxia silences expression of frataxin isoform E.
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- Scientific Reports, 2022, v. 12, n. 1, p. 1, doi. 10.1038/s41598-022-09002-5
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- Article
Iron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon?
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- Pharmaceuticals (14248247), 2018, v. 11, n. 3, p. 89, doi. 10.3390/ph11030089
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- Article
Reversible Axonal Dystrophy by Calcium Modulation in Frataxin-Deficient Sensory Neurons of YG8R Mice.
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- Frontiers in Molecular Neuroscience, 2017, p. 1, doi. 10.3389/fnmol.2017.00264
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- Article
Friedreich ataxia in Norway -- an epidemiological, molecular and clinical study.
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- Orphanet Journal of Rare Diseases, 2015, v. 10, n. 1, p. 1, doi. 10.1186/s13023-015-0328-4
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- Article
Neuroinflammation in Friedreich's Ataxia.
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- International Journal of Molecular Sciences, 2022, v. 23, n. 11, p. 6297, doi. 10.3390/ijms23116297
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Molecular Details of the Frataxin–Scaffold Interaction during Mitochondrial Fe–S Cluster Assembly.
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- International Journal of Molecular Sciences, 2021, v. 22, n. 11, p. 6006, doi. 10.3390/ijms22116006
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- Article
Future Prospects of Gene Therapy for Friedreich's Ataxia.
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- International Journal of Molecular Sciences, 2021, v. 22, n. 4, p. 1815, doi. 10.3390/ijms22041815
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- Article
Effects of Fe 2+ /Fe 3+ Binding to Human Frataxin and Its D122Y Variant, as Revealed by Site-Directed Spin Labeling (SDSL) EPR Complemented by Fluorescence and Circular Dichroism Spectroscopies.
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- International Journal of Molecular Sciences, 2020, v. 21, n. 24, p. 9619, doi. 10.3390/ijms21249619
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- Article
Effect of Mitochondrial and Cytosolic FXN Isoform Expression on Mitochondrial Dynamics and Metabolism.
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- International Journal of Molecular Sciences, 2020, v. 21, n. 21, p. 8251, doi. 10.3390/ijms21218251
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- Article
A Highly Conserved Iron-Sulfur Cluster Assembly Machinery between Humans and Amoeba Dictyostelium discoideum: The Characterization of Frataxin.
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- International Journal of Molecular Sciences, 2020, v. 21, n. 18, p. 6821, doi. 10.3390/ijms21186821
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- Article
The NRF2 Signaling Network Defines Clinical Biomarkers and Therapeutic Opportunity in Friedreich's Ataxia.
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- International Journal of Molecular Sciences, 2020, v. 21, n. 3, p. 916, doi. 10.3390/ijms21030916
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- Article
Nrf2-Inducers Counteract Neurodegeneration in Frataxin-Silenced Motor Neurons: Disclosing New Therapeutic Targets for Friedreich's Ataxia.
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- International Journal of Molecular Sciences, 2017, v. 18, n. 10, p. 2173, doi. 10.3390/ijms18102173
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- Article
Drosophila melanogaster Models of Metal-Related Human Diseases and Metal Toxicity.
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- International Journal of Molecular Sciences, 2017, v. 18, n. 7, p. 1456, doi. 10.3390/ijms18071456
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- Article
Liver Growth Factor (LGF) Upregulates Frataxin Protein Expression and Reduces Oxidative Stress in Friedreich's Ataxia Transgenic Mice.
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- International Journal of Molecular Sciences, 2016, v. 17, n. 12, p. 2066, doi. 10.3390/ijms17122066
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- Article
Frataxin Silencing Inactivates Mitochondrial Complex I in NSC34 Motoneuronal Cells and Alters Glutathione Homeostasis.
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- International Journal of Molecular Sciences, 2014, v. 15, n. 4, p. 5789, doi. 10.3390/ijms15045789
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- Article
Frataxin Deficiency Leads to Reduced Expression and Impaired Translocation of NF-E2-Related Factor (Nrf2) in Cultured Motor Neurons.
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- International Journal of Molecular Sciences, 2013, v. 14, n. 4, p. 7853, doi. 10.3390/ijms14047853
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- Article
Calcitriol increases frataxin levels and restores mitochondrial function in cell models of Friedreich Ataxia.
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- Biochemical Journal, 2021, v. 478, n. 1, p. 1, doi. 10.1042/BCJ20200331
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- Article
Frataxin-bypassing Isu1: characterization of the bypass activity in cells and mitochondria.
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- Biochemical Journal, 2014, v. 459, n. 1, p. 71, doi. 10.1042/BJ20131273
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- Article
Biochemistry of cardiomyopathy in the mitochondrial disease Friedreich's ataxia.
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- Biochemical Journal, 2013, v. 453, n. 3, p. 321, doi. 10.1042/BJ20130079
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- Article
Mesenchymal Stem Cell-Derived Factors Restore Function to Human Frataxin-Deficient Cells.
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- Cerebellum, 2017, v. 16, n. 4, p. 840, doi. 10.1007/s12311-017-0860-y
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- Article
Human Mesenchymal Stem Cells Increase Anti-oxidant Defences in Cells Derived from Patients with Friedreich's Ataxia.
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- Cerebellum, 2012, v. 11, n. 4, p. 861, doi. 10.1007/s12311-012-0406-2
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- Article
Effects of Erythropoietin on Frataxin Levels and Mitochondrial Function in Friedreich Ataxia - a Dose-Response Trial.
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- Cerebellum, 2011, v. 10, n. 4, p. 763, doi. 10.1007/s12311-011-0287-9
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- Article
The Neuropathology of Late-Onset Friedreich's Ataxia.
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- Cerebellum, 2011, v. 10, n. 1, p. 96, doi. 10.1007/s12311-010-0235-0
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- Article
Inducible and reversible phenotypes in a novel mouse model of Friedreich's Ataxia.
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- eLife, 2017, p. 1, doi. 10.7554/eLife.30054
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- Article
Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration.
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- eLife, 2016, p. 1, doi. 10.7554/eLife.16043
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- Article
Frataxin deficiency alters gene expression in Friedreich ataxia derived IPSC‐neurons and cardiomyocytes.
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- Molecular Genetics & Genomic Medicine, 2023, v. 11, n. 1, p. 1, doi. 10.1002/mgg3.2093
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- Article
DNA methylation in Friedreich ataxia silences expression of frataxin isoform E.
- Published in:
- Scientific Reports, 2022, v. 12, n. 1, p. 1, doi. 10.1038/s41598-022-09002-5
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- Publication type:
- Article
Protoporphyrin IX Binds to Iron(II)-Loaded and to Zinc-Loaded Human Frataxin.
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- Life (2075-1729), 2023, v. 13, n. 1, p. 222, doi. 10.3390/life13010222
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- Article
FRIEDREICH ATAXIA -- A CASE REPORT.
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- Nitte University Journal of Health Science, 2014, v. 4, n. 2, p. 133
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Friedreich Ataxia: Clinical Feature and Electrophysiological Symptoms.
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- Journal of Neurosciences in Rural Practice, 2017, v. 8, n. 4, p. 691, doi. 10.4103/jnrp.jnrp_248_17
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- Article
Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia.
- Published in:
- Nature Medicine, 2014, v. 20, n. 5, p. 542, doi. 10.1038/nm.3510
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- Article
Dimorphic frataxin and its gene regulation by sex steroids in hamsters.
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- Molecular Genetics & Genomics, 2023, v. 298, n. 3, p. 615, doi. 10.1007/s00438-023-02004-6
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- Article
Dorsal root ganglia in Friedreich ataxia: satellite cell proliferation and inflammation.
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- Acta Neuropathologica Communications, 2016, v. 4, p. 1, doi. 10.1186/s40478-016-0288-5
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- Article
The Cardioprotective Mechanism of Phenylaminoethyl Selenides (PAESe) Against Doxorubicin-Induced Cardiotoxicity Involves Frataxin.
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- Frontiers in Pharmacology, 2021, v. 11, p. N.PAG, doi. 10.3389/fphar.2020.574656
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- Article
Atypical Features in a Large Turkish Family Affected with Friedreich Ataxia.
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- Case Reports in Neurological Medicine, 2016, p. 1, doi. 10.1155/2016/4515938
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- Article
Frataxin Is Localized to Both the Chloroplast and Mitochondrion and Is Involved in Chloroplast Fe-S Protein Function in Arabidopsis.
- Published in:
- PLoS ONE, 2015, v. 10, n. 10, p. 1, doi. 10.1371/journal.pone.0141443
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On the Effect of Sodium Chloride and Sodium Sulfate on Cold Denaturation.
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- PLoS ONE, 2015, v. 10, n. 7, p. 1, doi. 10.1371/journal.pone.0133550
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- Article
Molecular Insights into Frataxin-Mediated Iron Supply for Heme Biosynthesis in Bacillus subtilis.
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- PLoS ONE, 2015, v. 10, n. 3, p. 1, doi. 10.1371/journal.pone.0122538
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- Article
The Pathogenesis of Cardiomyopathy in Friedreich Ataxia.
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- PLoS ONE, 2015, v. 10, n. 3, p. 1, doi. 10.1371/journal.pone.0116396
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- Article
Functional Characterization of Friedreich Ataxia iPS-Derived Neuronal Progenitors and Their Integration in the Adult Brain.
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- PLoS ONE, 2014, v. 9, n. 7, p. 1, doi. 10.1371/journal.pone.0101718
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- Article
Yeast Frataxin Is Stabilized by Low Salt Concentrations: Cold Denaturation Disentangles Ionic Strength Effects from Specific Interactions.
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- PLoS ONE, 2014, v. 9, n. 5, p. 1, doi. 10.1371/journal.pone.0095801
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- Article
Base Excision Repair of Chemotherapeutically-Induced Alkylated DNA Damage Predominantly Causes Contractions of Expanded GAA Repeats Associated with Friedreich's Ataxia.
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- PLoS ONE, 2014, v. 9, n. 4, p. 1, doi. 10.1371/journal.pone.0093464
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- Article
Bioenergetics of the Calf Muscle in Friedreich Ataxia Patients Measured by 31P-MRS Before and After Treatment with Recombinant Human Erythropoietin.
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- PLoS ONE, 2013, v. 8, n. 7, p. 1, doi. 10.1371/journal.pone.0069229
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- Article
Development of Frataxin Gene Expression Measures for the Evaluation of Experimental Treatments in Friedreich’s Ataxia
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- PLoS ONE, 2013, v. 8, n. 5, p. 1, doi. 10.1371/journal.pone.0063958
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- Article
Genetic Variations Creating MicroRNA Target Sites in the FXN 3'-UTR Affect Frataxin Expression in Friedreich Ataxia.
- Published in:
- PLoS ONE, 2013, v. 8, n. 1, p. 1, doi. 10.1371/journal.pone.0054791
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- Article