| scholarly article | Q13442814 |
| review article | Q7318358 |
| P50 | author | Aurélien Bayot | Q56863108 |
| P2093 | author name string | Pierre Rustin | |
| P2860 | cites work | Iron-sulfur protein maturation in human cells: evidence for a function of frataxin | Q24309526 |
| The GAA triplet-repeat expansion in Friedreich ataxia interferes with transcription and may be associated with an unusual DNA structure | Q24538667 | ||
| Hyperexpansion of GAA repeats affects post-initiation steps of FXN transcription in Friedreich's ataxia | Q24634126 | ||
| Therapeutic developments in Friedreich ataxia | Q26861733 | ||
| The role of mitochondria in cellular iron-sulfur protein biogenesis: mechanisms, connected processes, and diseases | Q26865882 | ||
| HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model | Q27301415 | ||
| Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin | Q27935734 | ||
| Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity | Q27937713 | ||
| Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS | Q28238983 | ||
| 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 | ||
| Clinical and genetic abnormalities in patients with Friedreich's ataxia | Q28290611 | ||
| The Friedreich's ataxia gene encodes a novel phosphatidylinositol-4- phosphate 5-kinase | Q28292228 | ||
| Two new pimelic diphenylamide HDAC inhibitors induce sustained frataxin upregulation in cells from Friedreich's ataxia patients and in a mouse model | Q28472583 | ||
| Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia | Q28474531 | ||
| Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues | Q28586434 | ||
| Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model | Q28743986 | ||
| Genotype and phenotype analysis of Friedreich's ataxia compound heterozygous patients | Q58184636 | ||
| DNA triplet repeats mediate heterochromatin-protein-1-sensitive variegated gene silencing | Q59071144 | ||
| Frataxin fracas | Q62959509 | ||
| Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features | Q70901775 | ||
| Disabled early recruitment of antioxidant defenses in Friedreich's ataxia | Q74599295 | ||
| Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells | Q77323923 | ||
| Quinone analogs prevent enzymes targeted in Friedreich ataxia from iron-induced injury in vitro | Q78027992 | ||
| Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study | Q78180596 | ||
| Epigenetic silencing in Friedreich ataxia is associated with depletion of CTCF (CCCTC-binding factor) and antisense transcription | Q33517116 | ||
| Friedreich's ataxia: the vicious circle hypothesis revisited | Q34045081 | ||
| Estrogen prevents oxidative damage to the mitochondria in Friedreich's ataxia skin fibroblasts | Q34235344 | ||
| Cis-silencing of PIP5K1B evidenced in Friedreich's ataxia patient cells results in cytoskeleton anomalies. | Q34336767 | ||
| Repeat expansion affects both transcription initiation and elongation in friedreich ataxia cells | Q34575655 | ||
| Small molecules affecting transcription in Friedreich ataxia | Q34582710 | ||
| Initial experience in the treatment of inherited mitochondrial disease with EPI-743. | Q34633928 | ||
| Friedreich's ataxia: pathology, pathogenesis, and molecular genetics | Q34708637 | ||
| Estrogen protection in Friedreich's ataxia skin fibroblasts | Q35046921 | ||
| The use of antioxidants in Friedreich's ataxia treatment | Q35094662 | ||
| The dual effect of Rac2 on phospholipase D2 regulation that explains both the onset and termination of chemotaxis | Q35096291 | ||
| Renal cyst formation in Fh1-deficient mice is independent of the Hif/Phd pathway: roles for fumarate in KEAP1 succination and Nrf2 signaling | Q35456356 | ||
| Long intronic GAA repeats causing Friedreich ataxia impede transcription elongation | Q36039174 | ||
| Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia | Q36488119 | ||
| Epigenetics in Friedreich's Ataxia: Challenges and Opportunities for Therapy | Q36663928 | ||
| A mitochondrial bioenergetic etiology of disease | Q36734995 | ||
| Epigenetics in nucleotide repeat expansion disorders | Q36848454 | ||
| Epigenetic changes and non-coding expanded repeats | Q37695480 | ||
| Fumarate hydratase inactivation in renal tumors: HIF1α, NRF2, and "cryptic targets" of transcription factors | Q38024881 | ||
| PPAR-gamma agonist Azelaoyl PAF increases frataxin protein and mRNA expression: new implications for the Friedreich's ataxia therapy. | Q39902235 | ||
| Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia. | Q40241487 | ||
| Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy | Q41932516 | ||
| Apn1 AP-endonuclease is essential for the repair of oxidatively damaged DNA bases in yeast frataxin-deficient cells. | Q41974951 | ||
| EGFR-activated signaling and actin remodeling regulate cyclic stretch-induced NRF2-ARE activation | Q42127192 | ||
| A cellular model for Friedreich Ataxia reveals small-molecule glutathione peroxidase mimetics as novel treatment strategy | Q42527600 | ||
| Rac1 and calmodulin interactions modulate dynamics of ARF6-dependent endocytosis. | Q42806256 | ||
| The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins | Q44092352 | ||
| Actin glutathionylation increases in fibroblasts of patients with Friedreich's ataxia: a potential role in the pathogenesis of the disease | Q44547310 | ||
| Idebenone treatment in Friedreich patients: one-year-long randomized placebo-controlled trial | Q44761938 | ||
| P275 | copyright license | Creative Commons Attribution 3.0 Unported | Q14947546 |
| P6216 | copyright status | copyrighted | Q50423863 |
| P921 | main subject | enzyme | Q8047 |
| transport protein | Q2111029 | ||
| metalloprotein | Q423350 | ||
| Friedreich ataxia | Q913856 | ||
| spinocerebellar degenerations | Q66124188 | ||
| P5008 | on focus list of Wikimedia project | ScienceSource | Q55439927 |
| P304 | page(s) | 725635 | |
| P577 | publication date | 2013-09-30 | |
| P1433 | published in | Oxidative medicine and cellular longevity | Q26840015 |
| P1476 | title | Friedreich's ataxia, frataxin, PIP5K1B: echo of a distant fracas | |
| P478 | volume | 2013 |
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