| 1 |
34801473 | Conformational stability, dynamics and function of human frataxin: Tryptophan side chain interplay. | 2022 Jan 15 |
1 |
| 2 |
34823116 | Molecular characteristics of proteins within the mitochondrial Fe-S cluster assembly complex. | 2022 Feb |
1 |
| 3 |
35079622 | FDX2 and ISCU Gene Variations Lead to Rhabdomyolysis With Distinct Severity and Iron Regulation. | 2022 Feb |
2 |
| 4 |
35203634 | Protein Mutations and Stability, a Link with Disease: The Case Study of Frataxin. | 2022 Feb 11 |
2 |
| 5 |
35221903 | Drug Repositioning in Friedreich Ataxia. | 2022 |
4 |
| 6 |
35310092 | Recent Advances in the Elucidation of Frataxin Biochemical Function Open Novel Perspectives for the Treatment of Friedreich's Ataxia. | 2022 |
13 |
| 7 |
35472330 | Posttranslational regulation of mitochondrial frataxin and identification of compounds that increase frataxin levels in Friedreich's ataxia. | 2022 Apr 25 |
6 |
| 8 |
35474632 | Towards a metabolomic approach to investigate iron-sulfur cluster biogenesis. | 2022 Apr 27 |
1 |
| 9 |
33272776 | A severe form of autosomal recessive spinocerebellar ataxia associated with novel PMPCA variants. | 2021 Mar |
1 |
| 10 |
33529321 | Defective palmitoylation of transferrin receptor triggers iron overload in Friedreich ataxia fibroblasts. | 2021 Apr 15 |
3 |
| 11 |
33672495 | Frataxins Emerge as New Players of the Intracellular Antioxidant Machinery. | 2021 Feb 20 |
1 |
| 12 |
33905372 | Frataxin deficiency promotes endothelial senescence in pulmonary hypertension. | 2021 Jun 1 |
4 |
| 13 |
33951329 | Drp1-dependent peptide reverse mitochondrial fragmentation, a homeostatic response in Friedreich ataxia. | 2021 May |
1 |
| 14 |
34046211 | Mitochondrial and metabolic dysfunction in Friedreich ataxia: update on pathophysiological relevance and clinical interventions. | 2021 Jun |
1 |
| 15 |
34199378 | Molecular Details of the Frataxin-Scaffold Interaction during Mitochondrial Fe-S Cluster Assembly. | 2021 Jun 2 |
2 |
| 16 |
34442352 | Neuro-Ophthalmological Findings in Friedreich's Ataxia. | 2021 Jul 23 |
1 |
| 17 |
34944579 | A Combined Spectroscopic and In Silico Approach to Evaluate the Interaction of Human Frataxin with Mitochondrial Superoxide Dismutase. | 2021 Nov 25 |
2 |
| 18 |
31343823 | Low frataxin mRNA expression is associated with inflammation and oxidative stress in patients with type 2 diabetes. | 2020 Jan |
2 |
| 19 |
31854120 | Age of onset determines intrinsic functional brain architecture in Friedreich ataxia. | 2020 Jan |
2 |
| 20 |
31877117 | Exenatide induces frataxin expression and improves mitochondrial function in Friedreich ataxia. | 2020 Jan 30 |
2 |
| 21 |
32019240 | The NRF2 Signaling Network Defines Clinical Biomarkers and Therapeutic Opportunity in Friedreich's Ataxia. | 2020 Jan 30 |
3 |
| 22 |
32169822 | Identification of Frataxin as a regulator of ferroptosis. | 2020 May |
7 |
| 23 |
32462051 | CRISPR-Cas9 Gene Editing of Hematopoietic Stem Cells from Patients with Friedreich's Ataxia. | 2020 Jun 12 |
4 |
| 24 |
32582297 | HMTase Inhibitors as a Potential Epigenetic-Based Therapeutic Approach for Friedreich's Ataxia. | 2020 |
1 |
| 25 |
32707090 | Relationship between activity and stability: Design and characterization of stable variants of human frataxin. | 2020 Sep 30 |
2 |
| 26 |
32739593 | Antioxidant defense mechanisms and its dysfunctional regulation in the mitochondrial disease, Friedreich's ataxia. | 2020 Nov 1 |
1 |
| 27 |
32909841 | Emerging therapies in Friedreich's Ataxia. | 2020 Dec |
2 |
| 28 |
32989015 | Loss of Mitochondrial Localization of Human FANCG Causes Defective FANCJ Helicase. | 2020 Nov 6 |
2 |
| 29 |
33126466 | An Overview of the Ferroptosis Hallmarks in Friedreich's Ataxia. | 2020 Oct 28 |
3 |
| 30 |
33214825 | Antiferroptotic Activity of Phenothiazine Analogues: A Novel Therapeutic Strategy for Oxidative Stress Related Disease. | 2020 Nov 12 |
1 |
| 31 |
33263002 | Molecular Defects in Friedreich's Ataxia: Convergence of Oxidative Stress and Cytoskeletal Abnormalities. | 2020 |
10 |
| 32 |
33348670 | Effects of Fe2+/Fe3+ Binding to Human Frataxin and Its D122Y Variant, as Revealed by Site-Directed Spin Labeling (SDSL) EPR Complemented by Fluorescence and Circular Dichroism Spectroscopies. | 2020 Dec 17 |
2 |
| 33 |
29958932 | Misdistribution of iron and oxidative stress in chronic kidney disease. | 2019 Mar |
3 |
| 34 |
30342111 | Ferrochelatase activity of plant frataxin. | 2019 Jan |
4 |
| 35 |
30635474 | Ferroptosis as a Novel Therapeutic Target for Friedreich's Ataxia. | 2019 Apr |
2 |
| 36 |
30636112 | Structural and functional characterization of a frataxin from a thermophilic organism. | 2019 Feb |
3 |
| 37 |
30778428 | The role of mitochondrial labile iron in Friedreich's ataxia skin fibroblasts sensitivity to ultraviolet A. | 2019 Mar 20 |
3 |
| 38 |
30833885 | The Role of Iron in Friedreich's Ataxia: Insights From Studies in Human Tissues and Cellular and Animal Models. | 2019 |
5 |
| 39 |
30874991 | Assessment of cell-free levels of iron and copper in patients with Friedreich's ataxia. | 2019 Apr |
2 |
| 40 |
30975898 | Mechanism of frataxin "bypass" in human iron-sulfur cluster biosynthesis with implications for Friedreich's ataxia. | 2019 Jun 7 |
7 |
| 41 |
31031004 | Hypoxia Rescues Frataxin Loss by Restoring Iron Sulfur Cluster Biogenesis. | 2019 May 30 |
1 |
| 42 |
31074541 | Characterization of human frataxin missense variants in cancer tissues. | 2019 Sep |
2 |
| 43 |
31101807 | Structure of the human frataxin-bound iron-sulfur cluster assembly complex provides insight into its activation mechanism. | 2019 May 17 |
3 |
| 44 |
31209948 | Evaluating the predictions of the protein stability change upon single amino acid substitutions for the FXN CAGI5 challenge. | 2019 Sep |
2 |
| 45 |
31317428 | Molecular Mechanisms and Therapeutics for the GAA·TTC Expansion Disease Friedreich Ataxia. | 2019 Oct |
1 |
| 46 |
31344531 | Exploring iron-binding to human frataxin and to selected Friedreich ataxia mutants by means of NMR and EPR spectroscopies. | 2019 Nov |
4 |
| 47 |
31395877 | Physiologically relevant reconstitution of iron-sulfur cluster biosynthesis uncovers persulfide-processing functions of ferredoxin-2 and frataxin. | 2019 Aug 8 |
4 |
| 48 |
31494282 | Friedreich ataxia- pathogenesis and implications for therapies. | 2019 Dec |
2 |
| 49 |
31511419 | Mechanism of activation of the human cysteine desulfurase complex by frataxin. | 2019 Sep 24 |
3 |
| 50 |
31584077 | SINEUP non-coding RNAs rescue defective frataxin expression and activity in a cellular model of Friedreich's Ataxia. | 2019 Nov 18 |
2 |