scholarly article | Q13442814 |
P819 | ADS bibcode | 2016PLoSO..1153574H |
P356 | DOI | 10.1371/JOURNAL.PONE.0153574 |
P3181 | OpenCitations bibliographic resource ID | 2307852 |
P932 | PMC publication ID | 4831832 |
P698 | PubMed publication ID | 27078885 |
P2093 | author name string | Genki Hayashi | |
Gino Cortopassi | |||
P2860 | cites work | Open-label pilot study of interferon gamma-1b in Friedreich ataxia | Q50458074 |
Targeting lipid peroxidation and mitochondrial imbalance in Friedreich's ataxia. | Q51615708 | ||
Constitutive expression of the human peroxiredoxin V gene contributes to protection of the genome from oxidative DNA lesions and to suppression of transcription of noncoding DNA | Q61794109 | ||
Dorsal root ganglion proteins in Friedreich's ataxia | Q72776794 | ||
Increased levels of plasma malondialdehyde in Friedreich ataxia | Q73289030 | ||
Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia | Q73295495 | ||
NADPH oxidase contributes directly to oxidative stress and apoptosis in nerve growth factor-deprived sympathetic neurons | Q73338159 | ||
Disabled early recruitment of antioxidant defenses in Friedreich's ataxia | Q74599295 | ||
Hyperexpansion of GAA repeats affects post-initiation steps of FXN transcription in Friedreich's ataxia | Q24634126 | ||
Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2−ΔΔCT Method | Q25938999 | ||
Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex | Q28115394 | ||
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 | ||
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 | ||
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 | ||
Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice | Q28512621 | ||
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 | ||
The domain organization of p67 phox, a protein required for activation of the superoxide-producing NADPH oxidase in phagocytes | Q30157433 | ||
Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia | Q30602174 | ||
Oxidative stress in patients with Friedreich ataxia. | Q31673183 | ||
Altered gene expression and DNA damage in peripheral blood cells from Friedreich's ataxia patients: cellular model of pathology | Q33525513 | ||
CLP36 interacts with palladin in dorsal root ganglion neurons | Q33551802 | ||
GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology | Q33743762 | ||
Clinical and genetic aspects of spinocerebellar degeneration | Q34021840 | ||
Human frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistry | Q34434217 | ||
Friedreich's ataxia: pathology, pathogenesis, and molecular genetics | Q34708637 | ||
Novel targets of sulforaphane in primary cardiomyocytes identified by proteomic analysis | Q35070877 | ||
Epigenetic therapy for Friedreich ataxia | Q35180607 | ||
Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxia | Q36129202 | ||
Evolution of the Friedreich's ataxia trinucleotide repeat expansion: founder effect and premutations | Q36658216 | ||
Surfactant protein D protects against acute hyperoxic lung injury | Q36935241 | ||
Mitochondrial peroxiredoxin involvement in antioxidant defence and redox signalling | Q37660501 | ||
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 | ||
Therapeutic approaches for the treatment of Friedreich's ataxia | Q39158402 | ||
Frataxin deficiency leads to reduced expression and impaired translocation of NF-E2-related factor (Nrf2) in cultured motor neurons | Q39168480 | ||
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 | ||
Mammalian frataxin directly enhances sulfur transfer of NFS1 persulfide to both ISCU and free thiols | Q41568940 | ||
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 | ||
Glutathione in blood of patients with Friedreich's ataxia. | Q43818051 | ||
Antioxidative stress effect of phosphoserine dimers is mediated via activation of the Nrf2 signaling pathway | Q46820640 | ||
P275 | copyright license | Creative Commons Attribution 4.0 International | Q20007257 |
P6216 | copyright status | copyrighted | Q50423863 |
P433 | issue | 4 | |
P407 | language of work or name | English | Q1860 |
P921 | main subject | biomarker | Q864574 |
Friedreich ataxia | Q913856 | ||
P304 | page(s) | e0153574 | |
P577 | publication date | 2016-01-01 | |
P1433 | published in | PLOS One | Q564954 |
P1476 | title | Lymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's Ataxia | |
P478 | volume | 11 |
Q47104232 | Comprehensive analysis of gene expression patterns in Friedreich's ataxia fibroblasts by RNA sequencing reveals altered levels of protein synthesis factors and solute carriers. |
Q63090502 | Safety, pharmacodynamics, and potential benefit of omaveloxolone in Friedreich ataxia |
Q89529709 | The NRF2 Signaling Network Defines Clinical Biomarkers and Therapeutic Opportunity in Friedreich's Ataxia |
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