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26635519 | Analyzing the Effects of a G137V Mutation in the FXN Gene. | 2015 |
1 |
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24138602 | Fixing frataxin: 'ironing out' the metabolic defect in Friedreich's ataxia. | 2014 Apr |
6 |
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24327207 | Modeling of Friedreich ataxia-related iron overloading cardiomyopathy using patient-specific-induced pluripotent stem cells. | 2014 Sep |
5 |
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24390816 | HFE p.C282Y heterozygosity is associated with earlier disease onset in Friedreich ataxia. | 2014 Jun |
2 |
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24429875 | The kinetics of folding of frataxin. | 2014 Apr 14 |
1 |
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24624085 | Mitochondrial iron-sulfur cluster dysfunction in neurodegenerative disease. | 2014 |
3 |
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24749505 | Cell and gene therapy for Friedreich ataxia: progress to date. | 2014 Aug |
3 |
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24792433 | Pathophysiogical and therapeutic progress in Friedreich ataxia. | 2014 May |
4 |
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24917819 | Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation. | 2014 |
6 |
| 110 |
24920569 | The alteration of the C-terminal region of human frataxin distorts its structural dynamics and function. | 2014 Aug |
2 |
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24971490 | Human frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistry. | 2014 Aug 5 |
11 |
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25034024 | Therapeutic approaches for the treatment of Friedreich's ataxia. | 2014 Aug |
4 |
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25113747 | Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia. | 2014 Dec 20 |
1 |
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25158131 | p53 directly regulates the transcription of the human frataxin gene and its lack of regulation in tumor cells decreases the utilization of mitochondrial iron. | 2014 Nov 1 |
6 |
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25228761 | Understanding the frustration arising from the competition between function, misfolding, and aggregation in a globular protein. | 2014 Sep 30 |
1 |
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25379420 | Cerebellar pathology in Friedreich's ataxia: atrophied dentate nuclei with normal iron content. | 2014 |
1 |
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22862424 | The p53-dependent expression of frataxin controls 5-aminolevulinic acid-induced accumulation of protoporphyrin IX and photo-damage in cancerous cells. | 2013 Jan-Feb |
3 |
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22917739 | Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia. | 2013 Jul |
9 |
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23136396 | Neurons and cardiomyocytes derived from induced pluripotent stem cells as a model for mitochondrial defects in Friedreich's ataxia. | 2013 May |
1 |
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23180044 | The mitochondrial protein frataxin is downregulated in hemodialysis patients. | 2013 Jun |
1 |
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23265191 | Mammalian frataxin controls sulfur production and iron entry during de novo Fe4S4 cluster assembly. | 2013 Jan 16 |
3 |
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23269675 | Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms. | 2013 Feb 8 |
9 |
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23334592 | Friedreich ataxia: neuropathology revised. | 2013 Feb |
2 |
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23574943 | Frataxin deficiency leads to reduced expression and impaired translocation of NF-E2-related factor (Nrf2) in cultured motor neurons. | 2013 Apr 10 |
2 |
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23859340 | Frataxin: a protein in search for a function. | 2013 Aug |
2 |
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23859348 | Co-enzyme Q10 and idebenone use in Friedreich's ataxia. | 2013 Aug |
1 |
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23859349 | Deferiprone for the treatment of Friedreich's ataxia. | 2013 Aug |
1 |
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23909240 | His86 from the N-terminus of frataxin coordinates iron and is required for Fe-S cluster synthesis. | 2013 Sep 3 |
16 |
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23936609 | Neurodegeneration in Friedreich's ataxia: from defective frataxin to oxidative stress. | 2013 |
2 |
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24100161 | Metabolic remodeling in frataxin-deficient yeast is mediated by Cth2 and Adr1. | 2013 Dec |
3 |
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25499576 | Insights into the role of oxidative stress in the pathology of Friedreich ataxia using peroxidation resistant polyunsaturated fatty acids. | 2013 |
2 |
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21936771 | Mutation in the Fe-S scaffold protein Isu bypasses frataxin deletion. | 2012 Jan 1 |
2 |
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22095894 | Exploring frataxin function. | 2012 Jan |
3 |
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22171070 | HSC20 interacts with frataxin and is involved in iron-sulfur cluster biogenesis and iron homeostasis. | 2012 Apr 1 |
3 |
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22200491 | Changes in mitochondrial glutathione levels and protein thiol oxidation in ∆yfh1 yeast cells and the lymphoblasts of patients with Friedreich's ataxia. | 2012 Feb |
2 |
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22352884 | Effector role reversal during evolution: the case of frataxin in Fe-S cluster biosynthesis. | 2012 Mar 27 |
8 |
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22399236 | Evidence for a role of frataxin in pancreatic islets isolated from multi-organ donors with and without type 2 diabetes mellitus. | 2012 Jun |
2 |
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22764179 | Cardiomyopathy in Friedreich ataxia: clinical findings and research. | 2012 Sep |
1 |
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22791549 | Therapeutic developments in Friedreich ataxia. | 2012 Sep |
5 |
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22859693 | Childhood ataxia: clinical features, pathogenesis, key unanswered questions, and future directions. | 2012 Sep |
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22897349 | Heterotrifunctional chemical cross-linking mass spectrometry confirms physical interaction between human frataxin and ISU. | 2012 Sep 4 |
3 |
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23049850 | Protein stability and dynamics modulation: the case of human frataxin. | 2012 |
2 |
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23115244 | Cells lacking pfh1, a fission yeast homolog of mammalian frataxin protein, display constitutive activation of the iron starvation response. | 2012 Dec 14 |
1 |
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23280845 | Central role and mechanisms of β-cell dysfunction and death in friedreich ataxia-associated diabetes. | 2012 Dec |
1 |
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23560311 | Characterising the neuropathology and neurobehavioural phenotype in Friedreich ataxia: a systematic review. | 2012 |
3 |
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20957368 | Expression, purification, and characterization of an iron chaperon protein CyaY from Acidithiobacillus ferrooxidans. | 2011 Mar |
3 |
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21147271 | Blood cells from Friedreich ataxia patients harbor frataxin deficiency without a loss of mitochondrial function. | 2011 Mar |
1 |
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21161302 | Iron-dependent functions of mitochondria--relation to neurodegeneration. | 2011 Mar |
5 |
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21298097 | Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex. | 2011 Jan 26 |
4 |
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21315377 | Friedreich's ataxia: pathology, pathogenesis, and molecular genetics. | 2011 Apr 15 |
4 |