| scholarly article | Q13442814 |
| P50 | author | David Alsina | Q84116041 |
| Rosa Purroy | Q91584588 | ||
| Jordi Tamarit | Q57062088 | ||
| P2093 | author name string | Joaquim Ros | |
| P2860 | cites work | Correlation between left ventricular hypertrophy and GAA trinucleotide repeat length in Friedreich's ataxia | Q73321027 |
| Sequence variation in GAA repeat expansions may cause differential phenotype display in Friedreich's ataxia | Q77365108 | ||
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| Yeast frataxin mutants display decreased superoxide dismutase activity crucial to promote protein oxidative damage | Q84938847 | ||
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| Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins | Q24303938 | ||
| In vivo maturation of human frataxin | Q24304209 | ||
| Frataxin interacts functionally with mitochondrial electron transport chain proteins | Q24305373 | ||
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| Assembly of human frataxin is a mechanism for detoxifying redox-active iron | Q24337696 | ||
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| Mechanisms of iron and copper-frataxin interactions. | Q50606747 | ||
| Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1). | Q51679611 | ||
| Interactions of iron-bound frataxin with ISCU and ferredoxin on the cysteine desulfurase complex leading to Fe-S cluster assembly. | Q52342683 | ||
| A structural approach to understanding the iron-binding properties of phylogenetically different frataxins. | Q54541223 | ||
| Friedreich's ataxia combined with diabetes mellitus in sisters. | Q55502210 | ||
| Friedreich's ataxia protein: phylogenetic evidence for mitochondrial dysfunction | Q56918650 | ||
| Evidence that yeast frataxin is not an iron storage protein in vivo | Q58031218 | ||
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| UCSF Chimera--a visualization system for exploratory research and analysis | Q27860666 | ||
| Yeast Frataxin Sequentially Chaperones and Stores Iron by Coupling Protein Assembly with Iron Oxidation | Q27930413 | ||
| Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase. | Q27932268 | ||
| Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia | Q27934042 | ||
| Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin | Q27935734 | ||
| An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1. | Q27939048 | ||
| Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity | Q27939599 | ||
| Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex | Q28115394 | ||
| Two-step processing of human frataxin by mitochondrial processing peptidase. Precursor and intermediate forms are cleaved at different rates | Q28140463 | ||
| Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS | Q28238983 | ||
| Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin | Q28244947 | ||
| Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes | Q28249379 | ||
| Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia | Q28250989 | ||
| Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion | Q28275699 | ||
| Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase | Q28279256 | ||
| Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia | Q28474531 | ||
| Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia | Q28646412 | ||
| Friedreich's ataxia, no changes in mitochondrial labile iron in human lymphoblasts and fibroblasts: a decrease in antioxidative capacity? | Q33210293 | ||
| The in vivo mitochondrial two-step maturation of human frataxin. | Q33363024 | ||
| Yeast frataxin solution structure, iron binding, and ferrochelatase interaction. | Q33808199 | ||
| Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex. | Q33813547 | ||
| Biophysical characterization of iron in mitochondria isolated from respiring and fermenting yeast | Q33966189 | ||
| Friedreich's ataxia: the vicious circle hypothesis revisited | Q34045081 | ||
| Normal and Friedreich Ataxia Cells Express Different Isoforms of Frataxin with Complementary Roles in Iron-Sulfur Cluster Assembly | Q34352285 | ||
| Human frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistry | Q34434217 | ||
| Novel frataxin isoforms may contribute to the pathological mechanism of Friedreich ataxia | Q34452330 | ||
| Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration. | Q34531992 | ||
| Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammals | Q34546241 | ||
| Co-precipitation of phosphate and iron limits mitochondrial phosphate availability in Saccharomyces cerevisiae lacking the yeast frataxin homologue (YFH1). | Q34675842 | ||
| Friedreich's ataxia: pathology, pathogenesis, and molecular genetics | Q34708637 | ||
| Version 4.0 of PaxDb: Protein abundance data, integrated across model organisms, tissues, and cell-lines. | Q35556314 | ||
| The Structure of the Complex between Yeast Frataxin and Ferrochelatase: CHARACTERIZATION AND PRE-STEADY STATE REACTION OF FERROUS IRON DELIVERY AND HEME SYNTHESIS. | Q35973559 | ||
| Metal Homeostasis Regulators Suppress FRDA Phenotypes in a Drosophila Model of the Disease | Q36081561 | ||
| Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia | Q36392948 | ||
| Relation of cytosolic iron excess to cardiomyopathy of Friedreich's ataxia | Q36438090 | ||
| Identification of nonferritin mitochondrial iron deposits in a mouse model of Friedreich ataxia | Q36483807 | ||
| The MCK mouse heart model of Friedreich's ataxia: Alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation | Q36775301 | ||
| Labile Low-Molecular-Mass Metal Complexes in Mitochondria: Trials and Tribulations of a Burgeoning Field | Q37309136 | ||
| His86 from the N-terminus of frataxin coordinates iron and is required for Fe-S cluster synthesis | Q37411896 | ||
| Friedreich ataxia: metal dysmetabolism in dorsal root ganglia | Q37485501 | ||
| Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question | Q37706874 | ||
| Frataxin: a protein in search for a function | Q38121942 | ||
| Deferiprone for the treatment of Friedreich's ataxia. | Q38121950 | ||
| Metabolic remodeling in frataxin-deficient yeast is mediated by Cth2 and Adr1. | Q38312097 | ||
| Compound heterozygous FXN mutations and clinical outcome in friedreich ataxia | Q38679540 | ||
| No changes in heme synthesis in human Friedreich´s ataxia erythroid progenitor cells | Q38835520 | ||
| Oxidative stress and altered lipid metabolism in Friedreich ataxia | Q38863980 | ||
| Frataxin deficiency leads to reduced expression and impaired translocation of NF-E2-related factor (Nrf2) in cultured motor neurons | Q39168480 | ||
| Iron-sulfur cluster biogenesis and trafficking in mitochondria | Q39374100 | ||
| Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation | Q39939146 | ||
| Redistribution of accumulated cell iron: a modality of chelation with therapeutic implications | Q40059081 | ||
| Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts | Q40209576 | ||
| Frataxin Depletion in Yeast Triggers Up-regulation of Iron Transport Systems before Affecting Iron-Sulfur Enzyme Activities | Q41466989 | ||
| Iron regulatory protein 1 sustains mitochondrial iron loading and function in frataxin deficiency. | Q41480174 | ||
| Mammalian frataxin directly enhances sulfur transfer of NFS1 persulfide to both ISCU and free thiols | Q41568940 | ||
| Nitric oxide prevents Aft1 activation and metabolic remodeling in frataxin-deficient yeast. | Q41694820 | ||
| Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer | Q41762099 | ||
| Cells lacking pfh1, a fission yeast homolog of mammalian frataxin protein, display constitutive activation of the iron starvation response | Q41892544 | ||
| The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxia | Q42176216 | ||
| Binding of yeast frataxin to the scaffold for Fe-S cluster biogenesis, Isu | Q42257695 | ||
| The factors governing the thermal stability of frataxin orthologues: how to increase a protein's stability | Q42627762 | ||
| Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model | Q42678097 | ||
| Cardiac energetics are abnormal in Friedreich ataxia patients in the absence of cardiac dysfunction and hypertrophy: an in vivo 31P magnetic resonance spectroscopy study. | Q43737617 | ||
| Apoptotic cell death and altered calcium homeostasis caused by frataxin depletion in dorsal root ganglia neurons can be prevented by BH4 domain of Bcl-xL protein | Q44649914 | ||
| Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia | Q44803998 | ||
| Deferiprone and idebenone rescue frataxin depletion phenotypes in a Drosophila model of Friedreich's ataxia | Q45258865 | ||
| Manganese is the link between frataxin and iron-sulfur deficiency in the yeast model of Friedreich ataxia. | Q46970707 | ||
| Causative role of oxidative stress in a Drosophila model of Friedreich ataxia. | Q47072048 | ||
| Mitoferrin modulates iron toxicity in a Drosophila model of Friedreich's ataxia. | Q47072104 | ||
| Iron-induced oligomerization of human FXN81-210 and bacterial CyaY frataxin and the effect of iron chelators. | Q47111560 | ||
| Frataxin-deficient neurons and mice models of Friedreich ataxia are improved by TAT-MTScs-FXN treatment | Q47693943 | ||
| Clinical Experience With Deferiprone Treatment for Friedreich Ataxia | Q47786184 | ||
| Biogenesis and functions of mammalian iron-sulfur proteins in the regulation of iron homeostasis and pivotal metabolic pathways | Q47862572 | ||
| FXN methylation predicts expression and clinical outcome in Friedreich ataxia. | Q47872898 | ||
| The expression of human mitochondrial ferritin rescues respiratory function in frataxin-deficient yeast | Q47949533 | ||
| The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteins | Q48197448 | ||
| Mitochondrial pore opening and loss of Ca2+ exchanger NCLX levels occur after frataxin depletion | Q48575301 | ||
| P275 | copyright license | Creative Commons Attribution 4.0 International | Q20007257 |
| P6216 | copyright status | copyrighted | Q50423863 |
| P433 | issue | 3 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | Friedreich ataxia | Q913856 |
| pathophysiology | Q1135939 | ||
| molecular medicine | Q3523816 | ||
| P304 | page(s) | 89 | |
| P577 | publication date | 2018-09-19 | |
| P1433 | published in | Pharmaceuticals | Q15750667 |
| P1476 | title | Iron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon? | |
| P478 | volume | 11 |
| Q93075404 | Emerging and Dynamic Biomedical Uses of Ferritin |
| Q91830287 | Frataxin-deficient cardiomyocytes present an altered thiol-redox state which targets actin and pyruvate dehydrogenase |
| Q91811029 | Iron as Therapeutic Target in Human Diseases |
| Q64076840 | Iron in Neurodegeneration - Cause or Consequence? |
| Q64040115 | The Role of Iron in Friedreich's Ataxia: Insights From Studies in Human Tissues and Cellular and Animal Models |
| Q64284099 | The role of mitochondrial labile iron in Friedreich's ataxia skin fibroblasts sensitivity to ultraviolet A |
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