scholarly article | Q13442814 |
P50 | author | Joel M. Gottesfeld | Q42410767 |
P2093 | author name string | Elisabetta Soragni | |
P2860 | cites work | SIRT1 regulates the histone methyl-transferase SUV39H1 during heterochromatin formation | Q24300487 |
Chemoproteomics profiling of HDAC inhibitors reveals selective targeting of HDAC complexes | Q24338109 | ||
The GAA triplet-repeat expansion in Friedreich ataxia interferes with transcription and may be associated with an unusual DNA structure | Q24538667 | ||
Hyperexpansion of GAA repeats affects post-initiation steps of FXN transcription in Friedreich's ataxia | Q24634126 | ||
Influence of Friedreich ataxia GAA noncoding repeat expansions on pre-mRNA processing | Q24647477 | ||
Iron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery | Q26850111 | ||
HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model | Q27301415 | ||
Histone Deacetylase (HDAC) Inhibitor Kinetic Rate Constants Correlate with Cellular Histone Acetylation but Not Transcription and Cell Viability | Q27679266 | ||
Epigenetic Therapeutics: A New Weapon in the War Against Cancer | Q28072370 | ||
Activating frataxin expression by repeat-targeted nucleic acids | Q28272901 | ||
Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion | Q28275699 | ||
Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes | Q28297330 | ||
Two new pimelic diphenylamide HDAC inhibitors induce sustained frataxin upregulation in cells from Friedreich's ataxia patients and in a mouse model | Q28472583 | ||
Oral administration of the pimelic diphenylamide HDAC inhibitor HDACi 4b is unsuitable for chronic inhibition of HDAC activity in the CNS in vivo | Q28483335 | ||
Development of frataxin gene expression measures for the evaluation of experimental treatments in Friedreich's ataxia | Q28488251 | ||
Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model | Q28743986 | ||
Trinucleotide Repeat Disorders | Q29038716 | ||
Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia | Q30602174 | ||
Chemical phylogenetics of histone deacetylases | Q30977637 | ||
Progressive GAA.TTC repeat expansion in human cell lines | Q33513958 | ||
Therapeutic application of histone deacetylase inhibitors for central nervous system disorders | Q37283454 | ||
Gene regulation and epigenetics in Friedreich's ataxia | Q38121941 | ||
Frataxin: a protein in search for a function | Q38121942 | ||
Deferiprone for the treatment of Friedreich's ataxia. | Q38121950 | ||
Transcriptional regulation by Polycomb group proteins | Q38149730 | ||
Histone variants: key players of chromatin | Q38208178 | ||
Pathophysiogical and therapeutic progress in Friedreich ataxia | Q38208992 | ||
Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation. | Q38219400 | ||
Epigenetic Treatment of Neurodegenerative Disorders: Alzheimer and Parkinson Diseases | Q38742951 | ||
Therapeutic approaches for the treatment of Friedreich's ataxia | Q39158402 | ||
Heterochromatinization induced by GAA-repeat hyperexpansion in Friedreich's ataxia can be reduced upon HDAC inhibition by vitamin B3. | Q39183027 | ||
The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues | Q40149163 | ||
Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia. | Q40241487 | ||
Improved Histone Deacetylase Inhibitors as Therapeutics for the Neurodegenerative Disease Friedreich's Ataxia: A New Synthetic Route | Q40783140 | ||
Pimelic diphenylamide 106 is a slow, tight-binding inhibitor of class I histone deacetylases | Q42109512 | ||
Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia | Q42226636 | ||
Mortality in Friedreich ataxia | Q43524683 | ||
Exploration of the internal cavity of histone deacetylase (HDAC) with selective HDAC1/HDAC2 inhibitors (SHI-1:2). | Q46817482 | ||
Discovery of indoles as potent and selective inhibitors of the deacetylase SIRT1. | Q46841189 | ||
Clinical Experience With Deferiprone Treatment for Friedreich Ataxia | Q47786184 | ||
Inhibitory effects of expanded GAA.TTC triplet repeats from intron I of the Friedreich ataxia gene on transcription and replication in vivo | Q48034382 | ||
Src inhibitors modulate frataxin protein levels. | Q53516540 | ||
DNA triplet repeats mediate heterochromatin-protein-1-sensitive variegated gene silencing | Q59071144 | ||
Preventing the ubiquitin–proteasome-dependent degradation of frataxin, the protein defective in Friedreich's ataxia | Q63090656 | ||
Sticky DNA, a self-associated complex formed at long GAA*TTC repeats in intron 1 of the frataxin gene, inhibits transcription | Q73842935 | ||
GAA instability in Friedreich's Ataxia shares a common, DNA-directed and intraallelic mechanism with other trinucleotide diseases | Q74771975 | ||
The high-resolution structure of the triplex formed by the GAA/TTC triplet repeat associated with Friedreich's ataxia | Q77928326 | ||
Breaking the silence in Friedreich's ataxia | Q80286140 | ||
Recombinant human erythropoietin increases frataxin protein expression without increasing mRNA expression | Q81527224 | ||
Epigenetic silencing in Friedreich ataxia is associated with depletion of CTCF (CCCTC-binding factor) and antisense transcription | Q33517116 | ||
The GAA*TTC triplet repeat expanded in Friedreich's ataxia impedes transcription elongation by T7 RNA polymerase in a length and supercoil dependent manner | Q33616633 | ||
Alleviating transcript insufficiency caused by Friedreich's ataxia triplet repeats | Q33857115 | ||
Chemical probes identify a role for histone deacetylase 3 in Friedreich's ataxia gene silencing | Q34019122 | ||
Altered nucleosome positioning at the transcription start site and deficient transcriptional initiation in Friedreich ataxia | Q34075853 | ||
Histone deacetylase complexes promote trinucleotide repeat expansions | Q34169694 | ||
Epigenetic promoter silencing in Friedreich ataxia is dependent on repeat length. | Q34315338 | ||
HDAC3-selective inhibitor enhances extinction of cocaine-seeking behavior in a persistent manner | Q34321375 | ||
Epigenetic and neurological effects and safety of high-dose nicotinamide in patients with Friedreich's ataxia: an exploratory, open-label, dose-escalation study. | Q34418695 | ||
Quantitative proteomic analysis identifies targets and pathways of a 2-aminobenzamide HDAC inhibitor in Friedreich's ataxia patient iPSC-derived neural stem cells. | Q34488569 | ||
Repeat expansion affects both transcription initiation and elongation in friedreich ataxia cells | Q34575655 | ||
Distinct pharmacological properties of second generation HDAC inhibitors with the benzamide or hydroxamate head group | Q34622291 | ||
Co-enzyme Q10 and idebenone use in Friedreich's ataxia | Q34654338 | ||
DNA sequence-specific polyamides alleviate transcription inhibition associated with long GAA.TTC repeats in Friedreich's ataxia | Q34984370 | ||
Mechanism of Action of 2-Aminobenzamide HDAC Inhibitors in Reversing Gene Silencing in Friedreich's Ataxia. | Q35149824 | ||
R-loops associated with triplet repeat expansions promote gene silencing in Friedreich ataxia and fragile X syndrome. | Q35160930 | ||
Epigenetic therapy for Friedreich ataxia | Q35180607 | ||
Activity-based probes for proteomic profiling of histone deacetylase complexes | Q35611605 | ||
A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse model | Q35770976 | ||
FXN Promoter Silencing in the Humanized Mouse Model of Friedreich Ataxia | Q35782974 | ||
Repeat-induced epigenetic changes in intron 1 of the frataxin gene and its consequences in Friedreich ataxia | Q35865338 | ||
Interferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model. | Q36026884 | ||
Long intronic GAA repeats causing Friedreich ataxia impede transcription elongation | Q36039174 | ||
Therapeutics development for triplet repeat expansion diseases | Q36277195 | ||
Expanded GAA repeats impede transcription elongation through the FXN gene and induce transcriptional silencing that is restricted to the FXN locus | Q36302082 | ||
Histone deacetylase (HDAC) inhibitors targeting HDAC3 and HDAC1 ameliorate polyglutamine-elicited phenotypes in model systems of Huntington's disease. | Q36482299 | ||
Mechanism-based profiling of enzyme families | Q36559644 | ||
Rating disease progression of Friedreich's ataxia by the International Cooperative Ataxia Rating Scale: analysis of a 603-patient database. | Q36760861 | ||
A gene expression phenotype in lymphocytes from Friedreich ataxia patients | Q36822492 | ||
In vivo imaging of pyrrole-imidazole polyamides with positron emission tomography | Q36861667 | ||
Reversal of epigenetic promoter silencing in Friedreich ataxia by a class I histone deacetylase inhibitor. | Q37021501 | ||
DNA triplexes and Friedreich ataxia | Q37062600 | ||
Rationale for the development of 2-aminobenzamide histone deacetylase inhibitors as therapeutics for Friedreich ataxia | Q37095593 | ||
Chemical probes for histone-modifying enzymes | Q37272504 | ||
P433 | issue | 9 | |
P921 | main subject | Friedreich ataxia | Q913856 |
P304 | page(s) | 961-970 | |
P577 | publication date | 2016-07-31 | |
P1433 | published in | Expert opinion on orphan drugs | Q27725467 |
P1476 | title | Translating HDAC inhibitors in Friedreich's ataxia | |
P478 | volume | 4 |
Q50003880 | Activation of Frataxin Protein Expression by Antisense Oligonucleotides Targeting the Mutant Expanded Repeat |
Q56700247 | Emerging therapeutics for the treatment of Friedreich’s ataxia |
Q92251738 | Exenatide induces frataxin expression and improves mitochondrial function in Friedreich ataxia |
Q47793738 | Overexpression of Drosophila frataxin triggers cell death in an iron-dependent manner. |
Q64245000 | Pharmacological Reactivation of the Silenced Gene as a Targeted Therapeutic Approach for Fragile X Syndrome |
Q92089407 | Progress in understanding Friedreich's ataxia using human induced pluripotent stem cells |
Q91173541 | Quantitating the epigenetic transformation contributing to cholesterol homeostasis using Gaussian process |
Q64040115 | The Role of Iron in Friedreich's Ataxia: Insights From Studies in Human Tissues and Cellular and Animal Models |
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