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Sexual dimorphism in a mouse model of Friedreich's ataxia with severe cardiomyopathy.
- Published in:
- Communications Biology, 2024, v. 7, n. 1, p. 1, doi. 10.1038/s42003-024-06962-4
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- Article
CRISPR/Cas9-based edition of frataxin gene in Dictyostelium discoideum.
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- Biochemical Journal, 2023, v. 480, n. 19, p. 1533, doi. 10.1042/BCJ20230244
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- Article
Expression and processing of mature human frataxin after gene therapy in mice.
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- Scientific Reports, 2024, v. 14, n. 1, p. 1, doi. 10.1038/s41598-024-59060-0
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- 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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- Publication type:
- Article
Transplantation of wild-type mouse hematopoietic stem and progenitor cells ameliorates deficits in a mouse model of Friedreich's ataxia.
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- Science Translational Medicine, 2017, v. 9, n. 413, p. 1, doi. 10.1126/scitranslmed.aaj2347
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- Article
SAXS and stability studies of iron-induced oligomers of bacterial frataxin CyaY.
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- PLoS ONE, 2017, v. 12, n. 9, p. 1, doi. 10.1371/journal.pone.0184961
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- Article
Progressive mitochondrial protein lysine acetylation and heart failure in a model of Friedreich’s ataxia cardiomyopathy.
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- PLoS ONE, 2017, v. 12, n. 5, p. 1, doi. 10.1371/journal.pone.0178354
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- Article
Disruption of Higher Order DNA Structures in Friedreich’s Ataxia (GAA)<sub>n</sub> Repeats by PNA or LNA Targeting.
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- PLoS ONE, 2016, v. 11, n. 11, p. 1, doi. 10.1371/journal.pone.0165788
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- Article
The mitochondrial protein frataxin is downregulated in hemodialysis patients.
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- Clinical & Experimental Nephrology, 2013, v. 17, n. 3, p. 424, doi. 10.1007/s10157-012-0737-9
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- Article
Low frataxin mRNA expression is associated with inflammation and oxidative stress in patients with type 2 diabetes.
- Published in:
- 2020
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- Publication type:
- journal article
Calcitriol increases frataxin levels and restores mitochondrial function in cell models of Friedreich Ataxia.
- Published in:
- 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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- Publication type:
- Article
Excision of Expanded GAA Repeats Alleviates the Molecular Phenotype of Friedreich's Ataxia.
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- Molecular Therapy, 2015, v. 23, n. 6, p. 1055, doi. 10.1038/mt.2015.41
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- Publication type:
- Article
Modelling of Friedreich's Ataxia and other Genetic Disorders as Defective / Noisy Genetic Information Processing.
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- Journal of Ultra Scientist of Physical Sciences - Section A (Mathematics), 2018, v. 30, n. 12, p. 409, doi. 10.22147/jusps-A/301201
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- Publication type:
- Article
Structural and Functional Characterization of CreFH1, the Frataxin Homolog from Chlamydomonas reinhardtii.
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- Plants (2223-7747), 2022, v. 11, n. 15, p. 1931, doi. 10.3390/plants11151931
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- Publication type:
- Article
Distribution of frataxin in eye retina of normal mice and of transgenic R7E mice with retinal degeneration.
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- Journal of Evolutionary Biochemistry & Physiology, 2010, v. 46, n. 4, p. 414, doi. 10.1134/S0022093010040113
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- Article
Inducible and reversible phenotypes in a novel mouse model of Friedreich's Ataxia.
- Published in:
- eLife, 2017, p. 1, doi. 10.7554/eLife.30054
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- Publication type:
- Article
Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration.
- Published in:
- eLife, 2016, p. 1, doi. 10.7554/eLife.16043
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- Publication type:
- Article
DNA Base Damage Repair Crosstalks with Chromatin Structures to Contract Expanded GAA Repeats in Friedreich's Ataxia.
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- Biomolecules (2218-273X), 2024, v. 14, n. 7, p. 809, doi. 10.3390/biom14070809
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- Publication type:
- Article
Protective Role of Dioscin against Doxorubicin-Induced Chronic Cardiotoxicity: Insights from Nrf2-GPX4 Axis-Mediated Cardiac Ferroptosis.
- Published in:
- Biomolecules (2218-273X), 2024, v. 14, n. 4, p. 422, doi. 10.3390/biom14040422
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- Publication type:
- Article
The Effect of Positive Charge Distribution on the Cryoprotective Activity of Dehydrins.
- Published in:
- Biomolecules (2218-273X), 2022, v. 12, n. 10, p. N.PAG, doi. 10.3390/biom12101510
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- Publication type:
- Article
Probing the Kinetic Stabilities of Friedreich's Ataxia Clinical Variants Using a Solid Phase GroEL Chaperonin Capture Platform.
- Published in:
- Biomolecules (2218-273X), 2014, v. 4, n. 4, p. 956, doi. 10.3390/biom4040956
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- Article
Frataxin gene editing rescues Friedreich's ataxia pathology in dorsal root ganglia organoid-derived sensory neurons.
- Published in:
- Nature Communications, 2020, v. 11, n. 1, p. N.PAG, doi. 10.1038/s41467-020-17954-3
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- Article
Analyzing the Effects of a G137V Mutation in the FXN Gene.
- Published in:
- Frontiers in Molecular Neuroscience, 2015, v. 8, p. 1, doi. 10.3389/fnmol.2015.00066
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- Article
Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question.
- Published in:
- FASEB Journal, 2010, v. 24, n. 7, p. 2152, doi. 10.1096/fj.09-143222
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- Article
Friedreich's ataxia: the vicious circle hypothesis revisited.
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- BMC Medicine, 2011, v. 9, n. 1, p. 112, doi. 10.1186/1741-7015-9-112
- Publication type:
- Article
Structural Basis for Fe--S Cluster Assembly and tRNA Thiolation Mediated by IscS Protein--Protein Interactions.
- Published in:
- PLoS Biology, 2010, v. 8, n. 4, p. 1, doi. 10.1371/journal.pbio.1000354
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- Article
Modelling Protein Plasticity: The Example of Frataxin and Its Variants.
- Published in:
- Molecules, 2022, v. 27, n. 6, p. 1955, doi. 10.3390/molecules27061955
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- Article
Phenotypic variation of FXN compound heterozygotes in a Friedreich ataxia cohort.
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- Annals of Clinical & Translational Neurology, 2024, v. 11, n. 5, p. 1110, doi. 10.1002/acn3.52027
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- Article
Safety, pharmacokinetics, and pharmacodynamics of nomlabofusp (CTI‐1601) in Friedreich's ataxia.
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- Annals of Clinical & Translational Neurology, 2024, v. 11, n. 3, p. 540, doi. 10.1002/acn3.51971
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- Publication type:
- Article
Selected missense mutations impair frataxin processing in Friedreich ataxia.
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- Annals of Clinical & Translational Neurology, 2017, v. 4, n. 8, p. 575, doi. 10.1002/acn3.433
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- Publication type:
- Article
Frataxin levels in peripheral tissue in Friedreich ataxia.
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- Annals of Clinical & Translational Neurology, 2015, v. 2, n. 8, p. 831, doi. 10.1002/acn3.225
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- Publication type:
- 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
Identification of p38 MAPK as a novel therapeutic target for Friedreich’s ataxia.
- Published in:
- Scientific Reports, 2018, p. 1, doi. 10.1038/s41598-018-23168-x
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- Publication type:
- Article
Altered Expression of Mitoferrin and Frataxin, Larger Labile Iron Pool and Greater Mitochondrial DNA Damage in the Skeletal Muscle of Older Adults.
- Published in:
- Cells (2073-4409), 2020, v. 9, n. 12, p. 2579, doi. 10.3390/cells9122579
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- Article
FRIEDREICH ATAXIA -- A CASE REPORT.
- Published in:
- Nitte University Journal of Health Science, 2014, v. 4, n. 2, p. 133
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- Article
Mitochondrial iron transport and homeostasis in plants.
- Published in:
- Frontiers in Plant Science, 2013, v. 4, p. 1, doi. 10.3389/fpls.2013.00348
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- Article
Mitochondrial Iron Transport and Homeostasis in Plants.
- Published in:
- Frontiers in Plant Science, 2013, v. 4, p. 1, doi. 10.3389/fpls.2013.00348
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- Publication type:
- Article
The iron-sulfur cluster assembly machineries in plants: current knowledge and open questions.
- Published in:
- Frontiers in Plant Science, 2013, v. 4, p. 1, doi. 10.3389/fpls.2013.00259
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- Publication type:
- Article
Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation.
- Published in:
- Frontiers in Pharmacology, 2014, v. 5, p. 1, doi. 10.3389/fphar.2014.00130
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- Publication type:
- Article
Biophysical characterisation of the recombinant human frataxin precursor.
- Published in:
- FEBS Open Bio, 2018, v. 8, n. 3, p. 390, doi. 10.1002/2211-5463.12376
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- Publication type:
- Article
The role of the N-terminal tail for the oligomerization, folding and stability of human frataxin.
- Published in:
- FEBS Open Bio, 2013, v. 3, p. 310, doi. 10.1016/j.fob.2013.07.004
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- Publication type:
- Article
The role of the N‐terminal tail for the oligomerization, folding and stability of human frataxin.
- Published in:
- FEBS Open Bio, 2013, v. 3, n. 1, p. 310, doi. 10.1016/j.fob.2013.07.004
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- Publication type:
- Article
Bone marrow transplantation stimulates neural repair in Friedreich's ataxia mice.
- Published in:
- 2018
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- Publication type:
- journal article
Frataxin and the molecular mechanism of mitochondrial iron-loading in Friedreich's ataxia.
- Published in:
- Clinical Science, 2016, v. 130, n. 11, p. 853, doi. 10.1042/CS20160072
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- Article
Frataxin: a protein in search for a function.
- Published in:
- Journal of Neurochemistry, 2013, v. 126, p. 43, doi. 10.1111/jnc.12220
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- Publication type:
- Article
Erythropoietin in Friedreich ataxia.
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- Journal of Neurochemistry, 2013, v. 126, p. 80, doi. 10.1111/jnc.12301
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- Publication type:
- Article
Deferiprone for the treatment of Friedreich's ataxia.
- Published in:
- Journal of Neurochemistry, 2013, v. 126, p. 142, doi. 10.1111/jnc.12300
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- Article
Cardiomyopathy of Friedreich Ataxia.
- Published in:
- Journal of Neurochemistry, 2013, v. 126, p. 88, doi. 10.1111/jnc.12217
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- Publication type:
- Article