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An In Silico Analysis of Genetic Variants and Structural Modeling of the Human Frataxin Protein in Friedreich's Ataxia.
- Published in:
- International Journal of Molecular Sciences, 2024, v. 25, n. 11, p. 5796, doi. 10.3390/ijms25115796
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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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- Publication type:
- 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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- Publication type:
- Article
Protective Role of Dioscin against Doxorubicin-Induced Chronic Cardiotoxicity: Insights from Nrf2-GPX4 Axis-Mediated Cardiac Ferroptosis.
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- Biomolecules (2218-273X), 2024, v. 14, n. 4, p. 422, doi. 10.3390/biom14040422
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- Publication type:
- Article
Frataxin analysis using triple quadrupole mass spectrometry: application to a large heterogeneous clinical cohort.
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- Journal of Neurology, 2024, v. 271, n. 4, p. 1844, doi. 10.1007/s00415-023-12118-x
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- Publication type:
- Article
A peptide derived from TID1S rescues frataxin deficiency and mitochondrial defects in FRDA cellular models.
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- Frontiers in Pharmacology, 2024, p. 1, doi. 10.3389/fphar.2024.1352311
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- Publication type:
- 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
Gene editing improves endoplasmic reticulum-mitochondrial contacts and unfolded protein response in Friedreich’s ataxia iPSC-derived neurons.
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- Frontiers in Pharmacology, 2024, p. 1, doi. 10.3389/fphar.2024.1323491
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- Publication type:
- Article
Emerging antioxidant therapies in Friedreich's ataxia.
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- Frontiers in Pharmacology, 2024, p. 1, doi. 10.3389/fphar.2024.1359618
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- Publication type:
- Article
Searching for Frataxin Function: Exploring the Analogy with Nqo15, the Frataxin-like Protein of Respiratory Complex I from Thermus thermophilus.
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- International Journal of Molecular Sciences, 2024, v. 25, n. 3, p. 1912, doi. 10.3390/ijms25031912
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- Publication type:
- Article
Quantification of human mature frataxin protein expression in nonhuman primate hearts after gene therapy.
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- Communications Biology, 2023, v. 6, n. 1, p. 1, doi. 10.1038/s42003-023-05472-z
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- Publication type:
- 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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- Publication type:
- Article
Rationale and protocol of a double-blind, randomized, placebo-controlled trial to test the efficacy, safety, and tolerability of dimethyl fumarate in Friedreich Ataxia (DMF-FA-201).
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- Frontiers in Neuroscience, 2023, p. 01, doi. 10.3389/fnins.2023.1260977
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- Publication type:
- Article
PPAR-gamma agonist pioglitazone recovers mitochondrial quality control in fibroblasts from PITRM1-deficient patients .
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- Frontiers in Pharmacology, 2023, p. 1, doi. 10.3389/fphar.2023.1220620
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- Publication type:
- 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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- Publication type:
- 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
Genome-Wide Identification, Characterization and Expression Profiling of Potato (Solanum tuberosum) Frataxin (FH) Gene.
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- Genes, 2023, v. 14, n. 2, p. 468, doi. 10.3390/genes14020468
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- Publication type:
- Article
A new FRDA mouse model [Fxn<sup>null</sup>:YG8s(GAA) > 800] with more than 800 GAA repeats.
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- Frontiers in Neuroscience, 2023, v. 17, p. 1, doi. 10.3389/fnins.2023.930422
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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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- Publication type:
- 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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- Publication type:
- 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
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
FECH Expression Correlates with the Prognosis and Tumor Immune Microenvironment in Clear Cell Renal Cell Carcinoma.
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- Journal of Oncology, 2022, p. 1, doi. 10.1155/2022/8943643
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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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- Article
Towards a metabolomic approach to investigate iron–sulfur cluster biogenesis.
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- IUBMB Life, 2022, v. 74, n. 7, p. 715, doi. 10.1002/iub.2618
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- Publication type:
- Article
Frataxin Deficit Leads to Reduced Dynamics of Growth Cones in Dorsal Root Ganglia Neurons of Friedreich's Ataxia YG8sR Model: A Multilinear Algebra Approach.
- Published in:
- Frontiers in Molecular Neuroscience, 2022, v. 15, p. 1, doi. 10.3389/fnmol.2022.912780
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- Publication type:
- 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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- Publication type:
- Article
Simultaneous Quantification of Mitochondrial Mature Frataxin and Extra-Mitochondrial Frataxin Isoform E in Friedreich's Ataxia Blood.
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- Frontiers in Neuroscience, 2022, v. 16, p. 1, doi. 10.3389/fnins.2022.874768
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- Publication type:
- 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
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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- Publication type:
- Article
Recent Advances in the Elucidation of Frataxin Biochemical Function Open Novel Perspectives for the Treatment of Friedreich's Ataxia.
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- Frontiers in Neuroscience, 2022, v. 16, p. 1, doi. 10.3389/fnins.2022.838335
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- Article
Mitochondrial De Novo Assembly of Iron–Sulfur Clusters in Mammals: Complex Matters in a Complex That Matters.
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- Inorganics, 2022, v. 10, n. 3, p. 31, doi. 10.3390/inorganics10030031
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- Publication type:
- Article
Selected Histone Deacetylase Inhibitors Reverse the Frataxin Transcriptional Defect in a Novel Friedreich's Ataxia Induced Pluripotent Stem Cell-Derived Neuronal Reporter System.
- Published in:
- Frontiers in Neuroscience, 2022, v. 16, p. 1, doi. 10.3389/fnins.2022.836476
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- Article
Drug Repositioning in Friedreich Ataxia.
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- Frontiers in Neuroscience, 2022, v. 16, p. 1, doi. 10.3389/fnins.2022.814445
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- Article
Protein Mutations and Stability, a Link with Disease: The Case Study of Frataxin.
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- Biomedicines, 2022, v. 10, n. 2, p. 425, doi. 10.3390/biomedicines10020425
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- Publication type:
- Article
Mice harboring the FXN I151F pathological point mutation present decreased frataxin levels, a Friedreich ataxia-like phenotype, and mitochondrial alterations.
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- Cellular & Molecular Life Sciences, 2022, v. 79, n. 2, p. 1, doi. 10.1007/s00018-021-04100-5
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- Article
A Combined Spectroscopic and In Silico Approach to Evaluate the Interaction of Human Frataxin with Mitochondrial Superoxide Dismutase.
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- Biomedicines, 2021, v. 9, n. 12, p. 1763, doi. 10.3390/biomedicines9121763
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- Article
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
Frataxin deficiency promotes endothelial senescence in pulmonary hypertension.
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- 2021
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- Publication type:
- journal article
Frataxin and endothelial cell senescence in pulmonary hypertension.
- Published in:
- 2021
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- Publication type:
- journal article
The Cardioprotective Mechanism of Phenylaminoethyl Selenides (PAESe) Against Doxorubicin-Induced Cardiotoxicity Involves Frataxin.
- Published in:
- Frontiers in Pharmacology, 2021, v. 11, p. N.PAG, doi. 10.3389/fphar.2020.574656
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- Publication type:
- Article
Mitochondrial iron and calcium homeostasis in Friedreich ataxia.
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- IUBMB Life, 2021, v. 73, n. 3, p. 543, doi. 10.1002/iub.2457
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- Publication type:
- Article
Nuclear Factor Erythroid 2-Related Factor 2 Activation Might Mitigate Clinical Symptoms in Friedreich's Ataxia: Clues of an "Out-Brain Origin" of the Disease From a Family Study.
- Published in:
- Frontiers in Neuroscience, 2021, v. 14, p. N.PAG, doi. 10.3389/fnins.2021.638810
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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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- Publication type:
- Article
Frataxins Emerge as New Players of the Intracellular Antioxidant Machinery.
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- Antioxidants, 2021, v. 10, n. 2, p. 315, doi. 10.3390/antiox10020315
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- Article
Role of contactin-1 axonal glycoprotein and polyphenol in a mice model of friedreich ataxia.
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- Italian Journal of Anatomy & Embryology / Archivio Italiano di Anatomia Ed Embriologia, 2021, v. 125, p. 144
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- Publication type:
- 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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- Publication type:
- 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
Altered Expression of Mitoferrin and Frataxin, Larger Labile Iron Pool and Greater Mitochondrial DNA Damage in the Skeletal Muscle of Older Adults.
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- Cells (2073-4409), 2020, v. 9, n. 12, p. 2579, doi. 10.3390/cells9122579
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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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- Publication type:
- Article