FXN Promoter Silencing in the Humanized Mouse Model of Friedreich Ataxia

scientific article published on 22 September 2015

FXN Promoter Silencing in the Humanized Mouse Model of Friedreich Ataxia is …
instance of (P31):
scholarly articleQ13442814

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P819ADS bibcode2015PLoSO..1038437C
P356DOI10.1371/JOURNAL.PONE.0138437
P932PMC publication ID4579136
P698PubMed publication ID26393353
P5875ResearchGate publication ID282129867

P2093author name stringSanjay I Bidichandani
Whitney N Costello
Yogesh K Chutake
Mark A Pook
Christina C Lam
Aniruddha C Parikh
Michael G Michalopulos
Tamara T Hughes
P2860cites workThe GAA triplet-repeat expansion in Friedreich ataxia interferes with transcription and may be associated with an unusual DNA structureQ24538667
Hyperexpansion of GAA repeats affects post-initiation steps of FXN transcription in Friedreich's ataxiaQ24634126
Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansionQ28275699
Clinical and genetic abnormalities in patients with Friedreich's ataxiaQ28290611
Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse modelQ28743986
Housekeeping and tissue-specific genes in mouse tissuesQ33285439
Altered nucleosome positioning at the transcription start site and deficient transcriptional initiation in Friedreich ataxiaQ34075853
Epigenetic promoter silencing in Friedreich ataxia is dependent on repeat length.Q34315338
Human frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistryQ34434217
Repeat expansion affects both transcription initiation and elongation in friedreich ataxia cellsQ34575655
A novel GAA-repeat-expansion-based mouse model of Friedreich's ataxiaQ35144024
Epigenetic therapy for Friedreich ataxiaQ35180607
Repeat-induced epigenetic changes in intron 1 of the frataxin gene and its consequences in Friedreich ataxiaQ35865338
Long intronic GAA repeats causing Friedreich ataxia impede transcription elongationQ36039174
Rating disease progression of Friedreich's ataxia by the International Cooperative Ataxia Rating Scale: analysis of a 603-patient database.Q36760861
Friedreich ataxia clinical outcome measures: natural history evaluation in 410 participantsQ36907355
Reversal of epigenetic promoter silencing in Friedreich ataxia by a class I histone deacetylase inhibitor.Q37021501
Friedreich ataxia: neuropathology revisedQ37278583
Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia.Q40241487
The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxiaQ42176216
DNA methylation in intron 1 of the frataxin gene is related to GAA repeat length and age of onset in Friedreich ataxia patients.Q46520508
FXN methylation predicts expression and clinical outcome in Friedreich ataxia.Q47872898
Inhibitory effects of expanded GAA.TTC triplet repeats from intron I of the Friedreich ataxia gene on transcription and replication in vivoQ48034382
Methylation-sensitive high-resolution meltingQ57390838
DNA triplet repeats mediate heterochromatin-protein-1-sensitive variegated gene silencingQ59071144
Sticky DNA, a self-associated complex formed at long GAA*TTC repeats in intron 1 of the frataxin gene, inhibits transcriptionQ73842935
Mammalian frataxin controls sulfur production and iron entry during de novo Fe4S4 cluster assemblyQ85780392
P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P6216copyright statuscopyrightedQ50423863
P433issue9
P407language of work or nameEnglishQ1860
P921main subjectFriedreich ataxiaQ913856
P304page(s)e0138437
P577publication date2015-09-22
P1433published inPLOS OneQ564954
P1476titleFXN Promoter Silencing in the Humanized Mouse Model of Friedreich Ataxia
P478volume10

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cites work (P2860)
Q45863203Deletion of the GAA repeats from the human frataxin gene using the CRISPR-Cas9 system in YG8R-derived cells and mouse models of Friedreich ataxia
Q47120765Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia.
Q55394024Increased Frataxin Expression Induced in Friedreich Ataxia Cells by Platinum TALE-VP64s or Platinum TALE-SunTag.
Q37021501Reversal of epigenetic promoter silencing in Friedreich ataxia by a class I histone deacetylase inhibitor.
Q47339311The role of oxidative stress in Friedreich's ataxia
Q39230731Translating HDAC inhibitors in Friedreich's ataxia.

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