| scholarly article | Q13442814 |
| P819 | ADS bibcode | 2014PLoSO...9j7416A |
| P356 | DOI | 10.1371/JOURNAL.PONE.0107416 |
| P932 | PMC publication ID | 4157886 |
| P698 | PubMed publication ID | 25198290 |
| P5875 | ResearchGate publication ID | 265420122 |
| P2093 | author name string | Chiranjeevi Sandi | |
| Mark A Pook | |||
| Sahar Al-Mahdawi | |||
| Sara Anjomani Virmouni | |||
| P2860 | cites work | Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesis | Q77195827 |
| Friedreich ataxia: the clinical picture | Q37414025 | ||
| Clinical Use of Interferon-gamma | Q37675044 | ||
| 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 | ||
| Frataxin deficiency in pancreatic islets causes diabetes due to loss of beta cell mass | Q39814673 | ||
| The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues | Q40149163 | ||
| 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 | ||
| Human BAC-mediated rescue of the Friedreich ataxia knockout mutation in transgenic mice | Q45875661 | ||
| GAA repeat instability in Friedreich ataxia YAC transgenic mice. | Q47248481 | ||
| Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria | Q48047307 | ||
| Increased iron in the dentate nucleus of patients with Friedrich's ataxia | Q48166308 | ||
| The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteins | Q48197448 | ||
| The GAA triplet-repeat is unstable in the context of the human FXN locus and displays age-dependent expansions in cerebellum and DRG in a transgenic mouse model | Q48402943 | ||
| Frataxin knockin mouse | Q57950173 | ||
| Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia | Q73295495 | ||
| An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1. | Q27939048 | ||
| Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes | Q28249379 | ||
| Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion | Q28275699 | ||
| Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits | Q28505173 | ||
| Generation and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell models | Q28540063 | ||
| Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model | Q28743986 | ||
| Development of histone deacetylase inhibitors as therapeutics for neurological disease | Q33670661 | ||
| GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology | Q33743762 | ||
| Friedreich's ataxia: pathology, pathogenesis, and molecular genetics | Q34708637 | ||
| The molecular basis of Friedreich ataxia. | Q35075287 | ||
| Interferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model. | Q36026884 | ||
| IFN-gamma: recent advances in understanding regulation of expression, biological functions, and clinical applications. | Q36985366 | ||
| Central role and mechanisms of β-cell dysfunction and death in friedreich ataxia-associated diabetes | Q36987314 | ||
| Lateral-flow immunoassay for the frataxin protein in Friedreich's ataxia patients and carriers | Q37217378 | ||
| P275 | copyright license | Creative Commons Attribution 4.0 International | Q20007257 |
| P6216 | copyright status | copyrighted | Q50423863 |
| P433 | issue | 9 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | Friedreich ataxia | Q913856 |
| P304 | page(s) | e107416 | |
| P577 | publication date | 2014-09-08 | |
| P1433 | published in | PLOS One | Q564954 |
| P1476 | title | Cellular, molecular and functional characterisation of YAC transgenic mouse models of Friedreich ataxia | |
| P478 | volume | 9 |
| Q35144024 | A novel GAA-repeat-expansion-based mouse model of Friedreich's ataxia |
| Q53435286 | Bone marrow transplantation stimulates neural repair in Friedreich's ataxia mice. |
| Q45752950 | Characterization of the retinal pigment epithelium in Friedreich ataxia |
| Q37663926 | Cytokine therapy-mediated neuroprotection in a Friedreich's ataxia mouse model |
| Q45863203 | Deletion of the GAA repeats from the human frataxin gene using the CRISPR-Cas9 system in YG8R-derived cells and mouse models of Friedreich ataxia |
| Q35710278 | Identification of telomere dysfunction in Friedreich ataxia |
| Q36588584 | Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia |
| Q45956921 | Neurobehavioral deficits in the KIKO mouse model of Friedreich's ataxia. |
| Q46169576 | Transplantation of wild-type mouse hematopoietic stem and progenitor cells ameliorates deficits in a mouse model of Friedreich's ataxia. |
| Q37036157 | Two different pathogenic mechanisms, dying-back axonal neuropathy and pancreatic senescence, are present in the YG8R mouse model of Friedreich's ataxia |
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