Lymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's Ataxia

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Lymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's Ataxia is …
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scholarly articleQ13442814

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P819ADS bibcode2016PLoSO..1153574H
P356DOI10.1371/JOURNAL.PONE.0153574
P3181OpenCitations bibliographic resource ID2307852
P932PMC publication ID4831832
P698PubMed publication ID27078885

P2093author name stringGenki Hayashi
Gino Cortopassi
P2860cites workOpen-label pilot study of interferon gamma-1b in Friedreich ataxiaQ50458074
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 DNAQ61794109
Dorsal root ganglion proteins in Friedreich's ataxiaQ72776794
Increased levels of plasma malondialdehyde in Friedreich ataxiaQ73289030
Clinical, biochemical and molecular genetic correlations in Friedreich's ataxiaQ73295495
NADPH oxidase contributes directly to oxidative stress and apoptosis in nerve growth factor-deprived sympathetic neuronsQ73338159
Disabled early recruitment of antioxidant defenses in Friedreich's ataxiaQ74599295
Hyperexpansion of GAA repeats affects post-initiation steps of FXN transcription in Friedreich's ataxiaQ24634126
Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2−ΔΔCT MethodQ25938999
Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complexQ28115394
Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranesQ28249379
Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxiaQ28250989
Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansionQ28275699
Clinical and genetic abnormalities in patients with Friedreich's ataxiaQ28290611
Two new pimelic diphenylamide HDAC inhibitors induce sustained frataxin upregulation in cells from Friedreich's ataxia patients and in a mouse modelQ28472583
Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxiaQ28474531
Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in miceQ28512621
Generation and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell modelsQ28540063
Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse modelQ28743986
The domain organization of p67 phox, a protein required for activation of the superoxide-producing NADPH oxidase in phagocytesQ30157433
Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxiaQ30602174
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 pathologyQ33525513
CLP36 interacts with palladin in dorsal root ganglion neuronsQ33551802
GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathologyQ33743762
Clinical and genetic aspects of spinocerebellar degenerationQ34021840
Human frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistryQ34434217
Friedreich's ataxia: pathology, pathogenesis, and molecular geneticsQ34708637
Novel targets of sulforaphane in primary cardiomyocytes identified by proteomic analysisQ35070877
Epigenetic therapy for Friedreich ataxiaQ35180607
Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxiaQ36129202
Evolution of the Friedreich's ataxia trinucleotide repeat expansion: founder effect and premutationsQ36658216
Surfactant protein D protects against acute hyperoxic lung injuryQ36935241
Mitochondrial peroxiredoxin involvement in antioxidant defence and redox signallingQ37660501
The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosisQ38328542
Therapeutic approaches for the treatment of Friedreich's ataxiaQ39158402
Frataxin deficiency leads to reduced expression and impaired translocation of NF-E2-related factor (Nrf2) in cultured motor neuronsQ39168480
Frataxin deficiency in pancreatic islets causes diabetes due to loss of beta cell massQ39814673
The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissuesQ40149163
Mammalian frataxin directly enhances sulfur transfer of NFS1 persulfide to both ISCU and free thiolsQ41568940
Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse modelQ42678097
Glutathione in blood of patients with Friedreich's ataxia.Q43818051
Antioxidative stress effect of phosphoserine dimers is mediated via activation of the Nrf2 signaling pathwayQ46820640
P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P6216copyright statuscopyrightedQ50423863
P433issue4
P407language of work or nameEnglishQ1860
P921main subjectbiomarkerQ864574
Friedreich ataxiaQ913856
P304page(s)e0153574
P577publication date2016-01-01
P1433published inPLOS OneQ564954
P1476titleLymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's Ataxia
P478volume11

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cites work (P2860)
Q47104232Comprehensive analysis of gene expression patterns in Friedreich's ataxia fibroblasts by RNA sequencing reveals altered levels of protein synthesis factors and solute carriers.
Q63090502Safety, pharmacodynamics, and potential benefit of omaveloxolone in Friedreich ataxia
Q89529709The NRF2 Signaling Network Defines Clinical Biomarkers and Therapeutic Opportunity in Friedreich's Ataxia

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