Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model
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Friedreich's Ataxia: A Neuronal Point of View on the Oxidative Stress HypothesisEmerging functional cross-talk between the Keap1-Nrf2 system and mitochondriaGeneration and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell modelsLymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's AtaxiaDyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia.Cellular, molecular and functional characterisation of YAC transgenic mouse models of Friedreich ataxia.Frataxin deficiency increases cyclooxygenase 2 and prostaglandins in cell and animal models of Friedreich's ataxia.A novel GAA-repeat-expansion-based mouse model of Friedreich's ataxiaHighly specific ubiquitin-competing molecules effectively promote frataxin accumulation and partially rescue the aconitase defect in Friedreich ataxia cells.Mesenchymal stem cells improve motor functions and decrease neurodegeneration in ataxic mice.TORC1 Inhibition by Rapamycin Promotes Antioxidant Defences in a Drosophila Model of Friedreich's AtaxiaNeurodegeneration in Friedreich's ataxia: from defective frataxin to oxidative stressCytokine therapy-mediated neuroprotection in a Friedreich's ataxia mouse modelFixing frataxin: 'ironing out' the metabolic defect in Friedreich's ataxiaOxidative stress in inherited mitochondrial diseases.Nerves Control Redox Levels in Mature Tissues Through Schwann Cells and Hedgehog Signaling.Frataxin silencing inactivates mitochondrial Complex I in NSC34 motoneuronal cells and alters glutathione homeostasisFrataxin deficiency leads to reduced expression and impaired translocation of NF-E2-related factor (Nrf2) in cultured motor neuronsPharmacological therapeutics in Friedreich ataxia: the present state.Liver Growth Factor (LGF) Upregulates Frataxin Protein Expression and Reduces Oxidative Stress in Friedreich's Ataxia Transgenic Mice.Lentivirus-meditated frataxin gene delivery reverses genome instability in Friedreich ataxia patient and mouse model fibroblastsMesenchymal Stem Cell-Derived Factors Restore Function to Human Frataxin-Deficient Cells.Mitigation of myocardial ischemia-reperfusion injury via HIF-1α-frataxin signaling.Time-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia.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.Oxidative Stress: Mechanistic Insights into Inherited Mitochondrial Disorders and Parkinson's Disease.Friedreich ataxia-induced pluripotent stem cell-derived neurons show a cellular phenotype that is corrected by a benzamide HDAC inhibitor.Frataxin deficiency impairs mitochondrial biogenesis in cells, mice and humans.Comprehensive analysis of gene expression patterns in Friedreich's ataxia fibroblasts by RNA sequencing reveals altered levels of protein synthesis factors and solute carriers.Nrf2-Inducers Counteract Neurodegeneration in Frataxin-Silenced Motor Neurons: Disclosing New Therapeutic Targets for Friedreich's Ataxia.Frataxin silencing alters microtubule stability in motor neurons: implications for Friedreich's ataxia.Bone marrow transplantation stimulates neural repair in Friedreich's ataxia mice.Phenothiazine antioxidants increase mitochondrial biogenesis and frataxin levels in Friedreich's ataxia cellsEmerging Regulatory Role of Nrf2 in Iron, Heme, and Hemoglobin Metabolism in Physiology and DiseaseIron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon?
P2860
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P2860
Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model
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2013 nî lūn-bûn
@nan
2013年の論文
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2013年論文
@yue
2013年論文
@zh-hant
2013年論文
@zh-hk
2013年論文
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2013年論文
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2013年论文
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2013年论文
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name
Frataxin deficiency leads to d ...... eich's ataxia YG8R mouse model
@en
Frataxin deficiency leads to d ...... eich's ataxia YG8R mouse model
@nl
type
label
Frataxin deficiency leads to d ...... eich's ataxia YG8R mouse model
@en
Frataxin deficiency leads to d ...... eich's ataxia YG8R mouse model
@nl
prefLabel
Frataxin deficiency leads to d ...... eich's ataxia YG8R mouse model
@en
Frataxin deficiency leads to d ...... eich's ataxia YG8R mouse model
@nl
P2093
P2860
P356
P1476
Frataxin deficiency leads to d ...... eich's ataxia YG8R mouse model
@en
P2093
Earl E Carstens
Eleonora Napoli
Genki Hayashi
Gino A Cortopassi
Mark A Pook
Mirela Iodi Carstens
Robert A Schoenfeld
P2860
P304
P356
10.1089/ARS.2012.4537
P50
P577
2013-03-28T00:00:00Z