| scholarly article | Q13442814 |
| P50 | author | Des R. Richardson | Q37369291 |
| Michael Huang | Q59668270 | ||
| Darius Lane | Q64166188 | ||
| P2093 | author name string | A Anzovino | |
| P2860 | cites work | Targeting of a human iron-sulfur cluster assembly enzyme, nifs, to different subcellular compartments is regulated through alternative AUG utilization | Q22008653 |
| Antioxidants and other pharmacological treatments for Friedreich ataxia | Q24200235 | ||
| Towards a unifying, systems biology understanding of large-scale cellular death and destruction caused by poorly liganded iron: Parkinson’s, Huntington’s, Alzheimer’s, prions, bactericides, chemical toxicology and others as examples | Q24289511 | ||
| Distinct iron-sulfur cluster assembly complexes exist in the cytosol and mitochondria of human cells | Q24290463 | ||
| The effects of frataxin silencing in HeLa cells are rescued by the expression of human mitochondrial ferritin | Q24305002 | ||
| Hepcidin regulates cellular iron efflux by binding to ferroportin and inducing its internalization | Q24310115 | ||
| Subcellular compartmentalization of human Nfu, an iron-sulfur cluster scaffold protein, and its ability to assemble a [4Fe-4S] cluster | Q24311963 | ||
| Assembly of human frataxin is a mechanism for detoxifying redox-active iron | Q24337696 | ||
| Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis | Q24338570 | ||
| A mouse model of juvenile hemochromatosis | Q24532268 | ||
| Mitochondrial ferritin limits oxidative damage regulating mitochondrial iron availability: hypothesis for a protective role in Friedreich ataxia | Q24619770 | ||
| Molecular control of vertebrate iron metabolism: mRNA-based regulatory circuits operated by iron, nitric oxide, and oxidative stress | Q24630216 | ||
| Human iron-sulfur cluster assembly, cellular iron homeostasis, and disease | Q24634143 | ||
| Distinct mechanisms of ferritin delivery to lysosomes in iron-depleted and iron-replete cells | Q24634199 | ||
| Friedreich ataxia: the clinical picture | Q37414025 | ||
| The pathogenesis of Friedreich ataxia and the structure and function of frataxin | Q37414028 | ||
| Function and biogenesis of iron-sulphur proteins | Q37579052 | ||
| Hereditary sideroblastic anemias: pathophysiology, diagnosis, and treatment | Q37604586 | ||
| pH and the recycling of transferrin during receptor-mediated endocytosis | Q37609089 | ||
| Mitochondrial ferritin expression in adult mouse tissues | Q37643132 | ||
| Iron regulatory proteins: from molecular mechanisms to drug development | Q37705384 | ||
| Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question | Q37706874 | ||
| Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches. | Q37736127 | ||
| Brain iron metabolism and its perturbation in neurological diseases. | Q37784423 | ||
| Disorders of iron metabolism. Part 1: molecular basis of iron homoeostasis | Q37822995 | ||
| Mitochondrial mayhem: the mitochondrion as a modulator of iron metabolism and its role in disease | Q37872302 | ||
| Mammalian iron homeostasis in health and disease: uptake, storage, transport, and molecular mechanisms of action | Q38063952 | ||
| Biochemistry of cardiomyopathy in the mitochondrial disease Friedreich's ataxia. | Q38121189 | ||
| The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis | Q38328542 | ||
| Coordinate Regulation of Glutathione Biosynthesis and Release by Nrf2-Expressing Glia Potently Protects Neurons from Oxidative Stress | Q38355236 | ||
| Frataxin deficiency leads to reduced expression and impaired translocation of NF-E2-related factor (Nrf2) in cultured motor neurons | Q39168480 | ||
| Intracellular iron trafficking: role of cytosolic ligands. | Q39393397 | ||
| The iron-binding function of transferrin in iron metabolism | Q39616563 | ||
| Low molecular weight intracellular iron transport compounds | Q39634380 | ||
| Frataxin deficiency induces Schwann cell inflammation and death | Q39813005 | ||
| The Steap proteins are metalloreductases | Q24681553 | ||
| Modulation of bone morphogenetic protein signaling in vivo regulates systemic iron balance | Q24682390 | ||
| The role of mitochondria in cellular iron-sulfur protein biogenesis and iron metabolism | Q26851591 | ||
| Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular models | Q26860523 | ||
| Mitochondria and mitophagy: the yin and yang of cell death control | Q27028020 | ||
| Crystal structure of human frataxin | Q27625397 | ||
| Towards a structural understanding of Friedreich's ataxia: the solution structure of frataxin | Q27625459 | ||
| Yeast Frataxin Sequentially Chaperones and Stores Iron by Coupling Protein Assembly with Iron Oxidation | Q27930413 | ||
| Deletion of the mitochondrial carrier genes MRS3 and MRS4 suppresses mitochondrial iron accumulation in a yeast frataxin-deficient strain | Q27931400 | ||
| 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 | ||
| Acidic residues of yeast frataxin have an essential role in Fe-S cluster assembly | Q27939103 | ||
| Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: evidence that Yfh1p affects Fe-S cluster synthesis | Q27939556 | ||
| Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity | Q27939599 | ||
| Mutation of a putative mitochondrial iron transporter gene (ABC7) in X-linked sideroblastic anemia and ataxia (XLSA/A) | Q28117190 | ||
| Positional cloning of zebrafish ferroportin1 identifies a conserved vertebrate iron exporter | Q28145559 | ||
| Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS | Q28238983 | ||
| Microcytic anaemia mice have a mutation in Nramp2, a candidate iron transporter gene | Q28244982 | ||
| 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 | ||
| Competitive regulation of hepcidin mRNA by soluble and cell-associated hemojuvelin | Q28260085 | ||
| Systemic iron homeostasis and the iron-responsive element/iron-regulatory protein (IRE/IRP) regulatory network | Q28280607 | ||
| Two to tango: regulation of Mammalian iron metabolism | Q28287034 | ||
| Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia | Q28474531 | ||
| Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits | Q28505173 | ||
| A human mitochondrial ferritin encoded by an intronless gene | Q28513826 | ||
| Inflammation alters the expression of DMT1, FPN1 and hepcidin, and it causes iron accumulation in central nervous system cells | Q28572077 | ||
| The iron-sulfur cluster of iron regulatory protein 1 modulates the accessibility of RNA binding and phosphorylation sites | Q28574626 | ||
| Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues | Q28586434 | ||
| Mitoferrin is essential for erythroid iron assimilation | Q28587125 | ||
| Identification of a ferrireductase required for efficient transferrin-dependent iron uptake in erythroid cells | Q28594532 | ||
| Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia | Q28646412 | ||
| Features of idebenone and related short-chain quinones that rescue ATP levels under conditions of impaired mitochondrial complex I | Q28730305 | ||
| Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model | Q28743986 | ||
| Mechanism of iron transport to the site of heme synthesis inside yeast mitochondria | Q77926867 | ||
| Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study | Q78180596 | ||
| Significance of mitochondria for porphyrin and heme biosynthesis | Q78532474 | ||
| Direct interorganellar transfer of iron from endosome to mitochondrion | Q80005581 | ||
| Iron-mediated dismutation of superoxide anion augments antigen-induced allergic inflammation: effect of lactoferrin | Q80347389 | ||
| Myocarditis and Friedreich's ataxia; a report of two cases | Q80619641 | ||
| Frataxin, a molecule of mystery: trading stability for function in its iron-binding site | Q82827948 | ||
| Idebenone in Friedreich ataxia cardiomyopathy-results from a 6-month phase III study (IONIA) | Q83582386 | ||
| The dorsal root ganglion in Friedreich's ataxia | Q84511482 | ||
| Rapamycin reduces oxidative stress in frataxin-deficient yeast cells | Q84587335 | ||
| Mammalian frataxin controls sulfur production and iron entry during de novo Fe4S4 cluster assembly | Q85780392 | ||
| Pioglitazone protects against renal ischemia-reperfusion injury by enhancing antioxidant capacity | Q86516862 | ||
| Autophagic degeneration as a possible mechanism of myocardial cell death in dilated cardiomyopathy | Q94029202 | ||
| Structure and function of ferrochelatase. | Q30417303 | ||
| Autosomal recessive cerebellar ataxias | Q31078351 | ||
| Friedreich's ataxia, no changes in mitochondrial labile iron in human lymphoblasts and fibroblasts: a decrease in antioxidative capacity? | Q33210293 | ||
| Deferiprone targets aconitase: implication for Friedreich's ataxia treatment | Q33344109 | ||
| PGC-1alpha down-regulation affects the antioxidant response in Friedreich's ataxia | Q33552305 | ||
| Iron redistribution as a therapeutic strategy for treating diseases of localized iron accumulation | Q33555894 | ||
| The iron transporter DMT1. | Q33785097 | ||
| Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex. | Q33813547 | ||
| Human frataxin: iron and ferrochelatase binding surface. | Q33821944 | ||
| Mitochondrial iron trafficking and the integration of iron metabolism between the mitochondrion and cytosol | Q33934515 | ||
| Friedreich's ataxia: the vicious circle hypothesis revisited | Q34045081 | ||
| Combinatorial mRNA regulation: iron regulatory proteins and iso-iron-responsive elements (Iso-IREs). | Q34076054 | ||
| Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities | Q34076650 | ||
| Mitochondrial ferritin expression in erythroid cells from patients with sideroblastic anemia | Q34156845 | ||
| Mitochondrial ferritin: a new player in iron metabolism | Q34172733 | ||
| Formation of iron-sulfur clusters in bacteria: an emerging field in bioinorganic chemistry. | Q34192764 | ||
| Stat5 regulates cellular iron uptake of erythroid cells via IRP-2 and TfR-1. | Q34293299 | ||
| Cis-silencing of PIP5K1B evidenced in Friedreich's ataxia patient cells results in cytoskeleton anomalies. | Q34336767 | ||
| Iron accumulation and iron-regulatory protein activity in human hepatoma (HepG2) cells | Q34367704 | ||
| Unique iron binding and oxidation properties of human mitochondrial ferritin: a comparative analysis with Human H-chain ferritin | Q34401195 | ||
| Deficiency of glutaredoxin 5 reveals Fe-S clusters are required for vertebrate haem synthesis | Q34444269 | ||
| Molecular bases of cellular iron toxicity | Q34615857 | ||
| Iron-sulfur cluster biogenesis and human disease | Q34656661 | ||
| Friedreich's ataxia: pathology, pathogenesis, and molecular genetics | Q34708637 | ||
| The labile iron pool: characterization, measurement, and participation in cellular processes(1). | Q34956696 | ||
| Friedreich's ataxia: iron chelators that target the mitochondrion as a therapeutic strategy? | Q35056066 | ||
| Over-expression of mitochondrial ferritin affects the JAK2/STAT5 pathway in K562 cells and causes mitochondrial iron accumulation | Q35265799 | ||
| Manganese superoxide dismutase: guardian of the powerhouse | Q35536092 | ||
| Frataxin, iron-sulfur clusters, heme, ROS, and aging. | Q35650906 | ||
| The metabolism of neuronal iron and its pathogenic role in neurological disease: review | Q35754462 | ||
| Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease | Q35794543 | ||
| HSC20 interacts with frataxin and is involved in iron-sulfur cluster biogenesis and iron homeostasis | Q35817466 | ||
| Iron trafficking in the mitochondrion: novel pathways revealed by disease. | Q35940415 | ||
| Activation of the HIF prolyl hydroxylase by the iron chaperones PCBP1 and PCBP2. | Q35993412 | ||
| DNA helicase and helicase-nuclease enzymes with a conserved iron-sulfur cluster | Q36044809 | ||
| Friedreich's ataxia causes redistribution of iron, copper, and zinc in the dentate nucleus | Q36398625 | ||
| Identification of nonferritin mitochondrial iron deposits in a mouse model of Friedreich ataxia | Q36483807 | ||
| The role of iron regulatory proteins in mammalian iron homeostasis and disease | Q36540433 | ||
| Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms | Q36596245 | ||
| Epigenetics in Friedreich's Ataxia: Challenges and Opportunities for Therapy | Q36663928 | ||
| The MCK mouse heart model of Friedreich's ataxia: Alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation | Q36775301 | ||
| A cytosolic iron chaperone that delivers iron to ferritin. | Q36826647 | ||
| Mammalian iron metabolism and its control by iron regulatory proteins | Q36910294 | ||
| Each member of the poly-r(C)-binding protein 1 (PCBP) family exhibits iron chaperone activity toward ferritin. | Q36929114 | ||
| Drug Insight: antioxidant therapy in inherited ataxias | Q37078851 | ||
| Friedreich ataxia: neuropathology revised | Q37278583 | ||
| Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant | Q37364051 | ||
| Autophagy in ischemic heart disease | Q37380461 | ||
| 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 | ||
| Sorting and recycling of cell surface receptors and endocytosed ligands: The asialoglycoprotein and transferrin receptors | Q40142274 | ||
| Evidence that inhibition of hemojuvelin shedding in response to iron is mediated through neogenin | Q40164674 | ||
| Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia. | Q40241487 | ||
| Overexpression of mitochondrial ferritin causes cytosolic iron depletion and changes cellular iron homeostasis. | Q40496469 | ||
| Erythroid differentiation and protoporphyrin IX down-regulate frataxin expression in Friend cells: characterization of frataxin expression compared to molecules involved in iron metabolism and hemoglobinization | Q40734827 | ||
| Human mitochondrial ferritin expressed in HeLa cells incorporates iron and affects cellular iron metabolism | Q40738268 | ||
| Tissue-specific regulation of iron metabolism and heme synthesis: distinct control mechanisms in erythroid cells. | Q41285748 | ||
| Differential modulation of the RNA-binding proteins IRP-1 and IRP-2 in response to iron. IRP-2 inactivation requires translation of another protein | Q41306409 | ||
| Binding of yeast frataxin to the scaffold for Fe-S cluster biogenesis, Isu | Q42257695 | ||
| Stat5 activation enables erythropoiesis in the absence of EpoR and Jak2. | Q42374469 | ||
| 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 | ||
| H(2)O(2) preferentially synergizes with nitroprusside to induce apoptosis associated with superoxide dismutase dysregulation in human melanoma irrespective of p53 status: Antagonism by o-phenanthroline. | Q42950706 | ||
| CCL7 and CXCL10 orchestrate oxidative stress-induced neutrophilic lung inflammation | Q43845879 | ||
| Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia | Q44031054 | ||
| Friedreich's ataxia: idebenone treatment in early stage patients | Q44171040 | ||
| Transferrin iron uptake is stimulated by ascorbate via an intracellular reductive mechanism. | Q44247856 | ||
| Transferrin Uptake by Rabbit Alveolar Macrophages in Vitro | Q44457554 | ||
| Acidification of endocytic compartments and the intracellular pathways of ligands and receptors | Q44950371 | ||
| Friedreich ataxia: the oxidative stress paradox | Q45198943 | ||
| Molecular and functional alterations in a mouse cardiac model of Friedreich ataxia: activation of the integrated stress response, eIF2α phosphorylation, and the induction of downstream targets. | Q47933778 | ||
| The expression of human mitochondrial ferritin rescues respiratory function in frataxin-deficient yeast | Q47949533 | ||
| Pro-inflammatory cytokines modulate iron regulatory protein 1 expression and iron transportation through reactive oxygen/nitrogen species production in ventral mesencephalic neurons | Q48179238 | ||
| The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteins | Q48197448 | ||
| Selective iron chelation in Friedreich ataxia: biologic and clinical implications | Q48231374 | ||
| Iron deficiency alters iron regulatory protein and iron transport protein expression in the perinatal rat brain | Q48372491 | ||
| Microglial activation in neuroinflammation: implications for the etiology of neurodegeneration | Q48678455 | ||
| Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1). | Q51679611 | ||
| Rat Schwann cells produce interleukin-1. | Q52235436 | ||
| Iron metabolism gene expression in human skeletal muscle | Q54416441 | ||
| A structural approach to understanding the iron-binding properties of phylogenetically different frataxins. | Q54541223 | ||
| Iron and iron-responsive proteins in the cardiomyopathy of Friedreich's ataxia | Q54975386 | ||
| The phylogenetic distribution of frataxin indicates a role in iron-sulfur cluster protein assembly | Q56879381 | ||
| Iron Regulatory Proteins Secure Mitochondrial Iron Sufficiency and Function | Q57397527 | ||
| Iron-responsive elements: regulatory RNA sequences that control mRNA levels and translation | Q57397582 | ||
| Evidence that yeast frataxin is not an iron storage protein in vivo | Q58031218 | ||
| Erythropoietin in Friedreich ataxia: No effect on frataxin in a randomized controlled trial | Q58451539 | ||
| A0001 in Friedreich ataxia: Biochemical characterization and effects in a clinical trial | Q60726008 | ||
| Erythropoietin in Friedreich ataxia: no effect on frataxin in a randomized controlled trial | Q64040323 | ||
| Mobilization of iron from endocytic vesicles. The effects of acidification and reduction | Q68750412 | ||
| Effect of pH and iron content of transferrin on its binding to reticulocyte receptors | Q70152410 | ||
| Distribution of iron in reticulocytes after inhibition of heme synthesis with succinylacetone: examination of the intermediates involved in iron metabolism | Q71010620 | ||
| Dietary iron intake modulates the activity of iron regulatory proteins and the abundance of ferritin and mitochondrial aconitase in rat liver | Q73087930 | ||
| Nutrient deprivation of cultured rat hepatocytes increases the desferrioxamine-available iron pool and augments the sensitivity to hydrogen peroxide | Q73692213 | ||
| Friedreich's ataxia | Q73702692 | ||
| Apoptotic versus autophagic cell death in heart failure | Q74257419 | ||
| TNF alpha, IFN gamma and IL-2 mRNA expression in CIDP sural nerve biopsies | Q77386593 | ||
| P433 | issue | 8 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | Friedreich ataxia | Q913856 |
| P304 | page(s) | 2174-2190 | |
| P577 | publication date | 2014-04-01 | |
| P1433 | published in | British Journal of Pharmacology | Q919631 |
| P1476 | title | Fixing frataxin: 'ironing out' the metabolic defect in Friedreich's ataxia | |
| P478 | volume | 171 |
| Q48623654 | Balance of cardiac and systemic hepcidin and its role in heart physiology and pathology |
| Q55073764 | Drosophila melanogaster Models of Friedreich's Ataxia. |
| Q37670668 | E3 Ligase RNF126 Directly Ubiquitinates Frataxin, Promoting Its Degradation: Identification of a Potential Therapeutic Target for Friedreich Ataxia. |
| Q49885816 | Hepcidin, an emerging and important player in brain iron homeostasis |
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| Q57954946 | Iron Oxides in the Human Brain |
| Q35115172 | Iron alters cell survival in a mitochondria-dependent pathway in ovarian cancer cells |
| Q92479421 | Large-scale contractions of Friedreich's ataxia GAA repeats in yeast occur during DNA replication due to their triplex-forming ability |
| Q41472664 | Mitigation of myocardial ischemia-reperfusion injury via HIF-1α-frataxin signaling |
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