| 51 |
31640150 | Targeting NRF2 for the Treatment of Friedreich's Ataxia: A Comparison among Drugs. | 2019 Oct 21 |
1 |
| 52 |
31939159 | Frataxin Structure and Function. | 2019 |
1 |
| 53 |
28782591 | Mitochondrial dysfunction in the neuro-degenerative and cardio-degenerative disease, Friedreich's ataxia. | 2018 Jul |
1 |
| 54 |
29090418 | Chemical shift assignment of a thermophile frataxin. | 2018 Apr |
1 |
| 55 |
29329987 | Quantitative proteomics in Friedreich's ataxia B-lymphocytes: A valuable approach to decipher the biochemical events responsible for pathogenesis. | 2018 Apr |
2 |
| 56 |
29342155 | Nanoscopic X-ray fluorescence imaging and quantification of intracellular key-elements in cryofrozen Friedreich's ataxia fibroblasts. | 2018 |
2 |
| 57 |
29406711 | ISCU(M108I) and ISCU(D39V) Differ from Wild-Type ISCU in Their Failure To Form Cysteine Desulfurase Complexes Containing Both Frataxin and Ferredoxin. | 2018 Mar 6 |
4 |
| 58 |
29491838 | Iron Sulfur and Molybdenum Cofactor Enzymes Regulate the Drosophila Life Cycle by Controlling Cell Metabolism. | 2018 |
1 |
| 59 |
29568068 | Identification of p38 MAPK as a novel therapeutic target for Friedreich's ataxia. | 2018 Mar 22 |
2 |
| 60 |
29572489 | Iron regulatory protein deficiency compromises mitochondrial function in murine embryonic fibroblasts. | 2018 Mar 23 |
2 |
| 61 |
29576242 | Interactions of iron-bound frataxin with ISCU and ferredoxin on the cysteine desulfurase complex leading to Fe-S cluster assembly. | 2018 Jun |
7 |
| 62 |
29596470 | Dysfunction in the mitochondrial Fe-S assembly machinery leads to formation of the chemoresistant truncated VDAC1 isoform without HIF-1α activation. | 2018 |
1 |
| 63 |
29794127 | Adding a temporal dimension to the study of Friedreich's ataxia: the effect of frataxin overexpression in a human cell model. | 2018 Jun 25 |
1 |
| 64 |
29983374 | Architectural Features of Human Mitochondrial Cysteine Desulfurase Complexes from Crosslinking Mass Spectrometry and Small-Angle X-Ray Scattering. | 2018 Aug 7 |
1 |
| 65 |
30031876 | Zinc(II) binding on human wild-type ISCU and Met140 variants modulates NFS1 desulfurase activity. | 2018 Sep |
4 |
| 66 |
30195758 | Increased Frataxin Expression Induced in Friedreich Ataxia Cells by Platinum TALE-VP64s or Platinum TALE-SunTag. | 2018 Sep 7 |
1 |
| 67 |
30235822 | Iron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon? | 2018 Sep 19 |
2 |
| 68 |
30519254 | Plant Frataxin in Metal Metabolism. | 2018 |
1 |
| 69 |
28271877 | The N-Terminus of Iron-Sulfur Cluster Assembly Factor ISD11 Is Crucial for Subcellular Targeting and Interaction with l-Cysteine Desulfurase NFS1. | 2017 Mar 28 |
1 |
| 70 |
28412459 | No changes in heme synthesis in human Friedreich´s ataxia erythroid progenitor cells. | 2017 Jul 20 |
1 |
| 71 |
28548666 | Zinc and the iron donor frataxin regulate oligomerization of the scaffold protein to form new Fe-S cluster assembly centers. | 2017 Jun 21 |
2 |
| 72 |
28634302 | Structure of human Fe-S assembly subcomplex reveals unexpected cysteine desulfurase architecture and acyl-ACP-ISD11 interactions. | 2017 Jul 3 |
4 |
| 73 |
28724340 | Pharmacological therapeutics in Friedreich ataxia: the present state. | 2017 Sep |
3 |
| 74 |
28882199 | Defining the Architecture of the Core Machinery for the Assembly of Fe-S Clusters in Human Mitochondria. | 2017 |
4 |
| 75 |
29125828 | Comprehensive analysis of gene expression patterns in Friedreich's ataxia fibroblasts by RNA sequencing reveals altered levels of protein synthesis factors and solute carriers. | 2017 Nov 1 |
1 |
| 76 |
29200434 | Iron-induced oligomerization of human FXN81-210 and bacterial CyaY frataxin and the effect of iron chelators. | 2017 |
6 |
| 77 |
29312931 | Identification of Gold Sensing Peptide by Integrative Proteomics and a Bacterial Two-Component System. | 2017 |
1 |
| 78 |
26589002 | Frataxin expression in reticulocytes of non-splenectomized and splenectomized patients with HbE-β-thalassaemia. | 2016 Apr |
2 |
| 79 |
26625322 | Efficient attenuation of Friedreich's ataxia (FRDA) cardiomyopathy by modulation of iron homeostasis-human induced pluripotent stem cell (hiPSC) as a drug screening platform for FRDA. | 2016 Jan 15 |
4 |
| 80 |
27019046 | Using human pluripotent stem cells to study Friedreich ataxia cardiomyopathy. | 2016 Jun 1 |
1 |
| 81 |
27029487 | Clinical Experience With Deferiprone Treatment for Friedreich Ataxia. | 2016 Jul |
1 |
| 82 |
27106929 | Time-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia. | 2016 May 15 |
1 |
| 83 |
27129098 | Frataxin and the molecular mechanism of mitochondrial iron-loading in Friedreich's ataxia. | 2016 Jun 1 |
5 |
| 84 |
27518705 | Lentivirus-meditated frataxin gene delivery reverses genome instability in Friedreich ataxia patient and mouse model fibroblasts. | 2016 Dec |
1 |
| 85 |
27901468 | Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammals. | 2016 Nov 30 |
2 |
| 86 |
28175303 | Friedreich ataxia-induced pluripotent stem cell-derived neurons show a cellular phenotype that is corrected by a benzamide HDAC inhibitor. | 2016 Nov 15 |
6 |
| 87 |
25533527 | A helix-coil transition induced by the metal ion interaction with a grafted iron-binding site of the CyaY protein family. | 2015 Feb 7 |
3 |
| 88 |
25597503 | Mammalian frataxin directly enhances sulfur transfer of NFS1 persulfide to both ISCU and free thiols. | 2015 Jan 19 |
2 |
| 89 |
25832196 | Structural characterization of metal binding to a cold-adapted frataxin. | 2015 Jun |
3 |
| 90 |
25929520 | Novel aberrant genetic and epigenetic events in Friedreich's ataxia. | 2015 Jul 1 |
3 |
| 91 |
25996492 | Turning Escherichia coli into a Frataxin-Dependent Organism. | 2015 May |
2 |
| 92 |
25996679 | Trading Places-Switching Frataxin Function by a Single Amino Acid Substitution within the [Fe-S] Cluster Assembly Scaffold. | 2015 May |
1 |
| 93 |
26016389 | The Human Iron-Sulfur Assembly Complex Catalyzes the Synthesis of [2Fe-2S] Clusters on ISCU2 That Can Be Transferred to Acceptor Molecules. | 2015 Jun 30 |
2 |
| 94 |
26016518 | Frataxin Accelerates [2Fe-2S] Cluster Formation on the Human Fe-S Assembly Complex. | 2015 Jun 30 |
9 |
| 95 |
26032732 | Interaction of frataxin, an iron binding protein, with IscU of Fe-S clusters biogenesis pathway and its upregulation in AmpB resistant Leishmania donovani. | 2015 Aug |
4 |
| 96 |
26138268 | Ultra-structural hair alterations in Friedreich's ataxia: A scanning electron microscopic investigation. | 2015 Aug |
1 |
| 97 |
26144971 | Iron-Starvation-Induced Mitophagy Mediates Lifespan Extension upon Mitochondrial Stress in C. elegans. | 2015 Jul 20 |
1 |
| 98 |
26286937 | Phenotypic Screening for Friedreich Ataxia Using Random shRNA Selection. | 2015 Oct |
3 |
| 99 |
26321457 | Investigation of mitochondrial DNA variations among Indian Friedreich's ataxia (FRDA) patients. | 2015 Nov |
2 |
| 100 |
26375377 | Friedreich Ataxia: From the Eye of a Molecular Biologist. | 2015 Sep |
1 |