Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model - Wikidata - wikimedia - TriplyDB

Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model

Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model

http://www.wikidata.org/entity/Q42678097

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Friedreich's Ataxia: A Neuronal Point of View on the Oxidative Stress Hypothesis Emerging functional cross-talk between the Keap1-Nrf2 system and mitochondria Generation and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell models Lymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's Ataxia Dyclonine 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 ataxia Highly 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 Ataxia Neurodegeneration in Friedreich's ataxia: from defective frataxin to oxidative stress Cytokine therapy-mediated neuroprotection in a Friedreich's ataxia mouse model Fixing frataxin: 'ironing out' the metabolic defect in Friedreich's ataxia Oxidative 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 homeostasis Frataxin deficiency leads to reduced expression and impaired translocation of NF-E2-related factor (Nrf2) in cultured motor neurons Pharmacological 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 fibroblasts Mesenchymal 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 cells Emerging Regulatory Role of Nrf2 in Iron, Heme, and Hemoglobin Metabolism in Physiology and Disease Iron in Friedreich Ataxia: A Central Role in the Pathophysiology or an Epiphenomenon?

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Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model

http://www.wikidata.org/entity/Q42678097

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Genki Hayashi

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P2860

Glutaredoxin 5 deficiency causes sideroblastic anemia by specifically impairing heme biosynthesis and depleting cytosolic iron in human erythroblasts

Iron-sulfur protein maturation in human cells: evidence for a function of frataxin

Nuclear transport, oxidative stress, and neurodegeneration

Splice mutation in the iron-sulfur cluster scaffold protein ISCU causes myopathy with exercise intolerance

Vitamin E, antioxidant and nothing more

Structure–Function Analysis of Friedreich’s Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe–S Assembly Complex

Glutathione-dependent redox status of frataxin-deficient cells in a yeast model of Friedreich's ataxia.

Diversity of structures and properties among catalases

Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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