| scholarly article | Q13442814 |
| P2093 | author name string | Joel M Gottesfeld | |
| Steve Jones | |||
| James R Rusche | |||
| Chiranjeevi Sandi | |||
| Mark A Pook | |||
| Sahar Al-Mahdawi | |||
| Ricardo Mouro Pinto | |||
| Glenn Barnes | |||
| Vahid Ezzatizadeh | |||
| P2860 | cites work | Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis | Q24615395 |
| The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice | Q24656405 | ||
| HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model | Q27301415 | ||
| The benzamide M344, a novel histone deacetylase inhibitor, significantly increases SMN2 RNA/protein levels in spinal muscular atrophy cells | Q28242279 | ||
| Clinical development of histone deacetylase inhibitors as anticancer agents | Q28244438 | ||
| 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 | ||
| LBH589 induces up to 10-fold SMN protein levels by several independent mechanisms and is effective even in cells from SMA patients non-responsive to valproate | Q28251155 | ||
| Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion | Q28275699 | ||
| Two new pimelic diphenylamide HDAC inhibitors induce sustained frataxin upregulation in cells from Friedreich's ataxia patients and in a mouse model | Q28472583 | ||
| Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits | Q28505173 | ||
| Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation | Q28510128 | ||
| Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia | Q28593887 | ||
| Histone deacetylase inhibitors | Q33210740 | ||
| Epigenetic silencing in Friedreich ataxia is associated with depletion of CTCF (CCCTC-binding factor) and antisense transcription | Q33517116 | ||
| Development of histone deacetylase inhibitors as therapeutics for neurological disease | Q33670661 | ||
| GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology | Q33743762 | ||
| Chemical probes identify a role for histone deacetylase 3 in Friedreich's ataxia gene silencing | Q34019122 | ||
| Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease | Q34763178 | ||
| Diagnosis and treatment of Friedreich ataxia: a European perspective | Q34974299 | ||
| Repeat-induced epigenetic changes in intron 1 of the frataxin gene and its consequences in Friedreich ataxia | Q35865338 | ||
| A persistent RNA.DNA hybrid formed by transcription of the Friedreich ataxia triplet repeat in live bacteria, and by T7 RNAP in vitro | Q36059529 | ||
| Histone deacetylase inhibitors as therapeutics for polyglutamine disorders | Q36599926 | ||
| Long intronic GAA*TTC repeats induce epigenetic changes and reporter gene silencing in a molecular model of Friedreich ataxia | Q36959663 | ||
| DNA triplexes and Friedreich ataxia | Q37062600 | ||
| The pathogenesis of Friedreich ataxia and the structure and function of frataxin | Q37414028 | ||
| New clinical developments in histone deacetylase inhibitors for epigenetic therapy of cancer | Q37503780 | ||
| Clinical studies of histone deacetylase inhibitors. | Q37512917 | ||
| Multiple mechanisms induce transcriptional silencing of a subset of genes, including oestrogen receptor alpha, in response to deacetylase inhibition by valproic acid and trichostatin A. | Q38326758 | ||
| 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 | ||
| The role of histone acetylation in SMN gene expression. | Q40437533 | ||
| Pimelic diphenylamide 106 is a slow, tight-binding inhibitor of class I histone deacetylases | Q42109512 | ||
| Dose-dependent blockade to cardiomyocyte hypertrophy by histone deacetylase inhibitors | Q44449807 | ||
| GAA repeat instability in Friedreich ataxia YAC transgenic mice. | Q47248481 | ||
| The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteins | Q48197448 | ||
| The GAA triplet-repeat is unstable in the context of the human FXN locus and displays age-dependent expansions in cerebellum and DRG in a transgenic mouse model | Q48402943 | ||
| In vitro and ex vivo evaluation of second-generation histone deacetylase inhibitors for the treatment of spinal muscular atrophy | Q48485610 | ||
| 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 | ||
| Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia | Q73295495 | ||
| Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesis | Q77195827 | ||
| Recombinant human erythropoietin increases frataxin protein expression without increasing mRNA expression | Q81527224 | ||
| The dorsal root ganglion in Friedreich's ataxia | Q84511482 | ||
| P433 | issue | 3 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | phenotype | Q104053 |
| Friedreich ataxia | Q913856 | ||
| P304 | page(s) | 496-505 | |
| P577 | publication date | 2011-06-01 | |
| P1433 | published in | Neurobiology of Disease | Q15716606 |
| P1476 | title | Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model | |
| P478 | volume | 42 |
| Q35144024 | A novel GAA-repeat-expansion-based mouse model of Friedreich's ataxia |
| Q36055920 | A role for epigenetic changes in the development of retinal neurodegenerative conditions |
| Q28272901 | Activating frataxin expression by repeat-targeted nucleic acids |
| Q50003880 | Activation of Frataxin Protein Expression by Antisense Oligonucleotides Targeting the Mutant Expanded Repeat |
| Q48670933 | Alleviating GAA Repeat Induced Transcriptional Silencing of the Friedreich's Ataxia Gene During Somatic Cell Reprogramming |
| Q38121944 | Animal and cellular models of Friedreich ataxia. |
| Q89781978 | Biochemical and Behavioral Characterization of IN14, a New Inhibitor of HDACs with Antidepressant-Like Properties |
| Q35670978 | Bromodomain inhibitors regulate the C9ORF72 locus in ALS |
| Q34150158 | Cellular, molecular and functional characterisation of YAC transgenic mouse models of Friedreich ataxia |
| Q45863203 | Deletion of the GAA repeats from the human frataxin gene using the CRISPR-Cas9 system in YG8R-derived cells and mouse models of Friedreich ataxia |
| Q28488251 | Development of frataxin gene expression measures for the evaluation of experimental treatments in Friedreich's ataxia |
| Q30602174 | Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia |
| Q92450855 | Efficient electroporation of neuronal cells using synthetic oligonucleotides: identifying duplex RNA and antisense oligonucleotide activators of human frataxin expression |
| Q34418695 | Epigenetic and neurological effects and safety of high-dose nicotinamide in patients with Friedreich's ataxia: an exploratory, open-label, dose-escalation study. |
| Q35180607 | Epigenetic therapy for Friedreich ataxia |
| Q38219414 | Epigenetic-based therapies for Friedreich ataxia |
| Q36663928 | Epigenetics in Friedreich's Ataxia: Challenges and Opportunities for Therapy |
| Q36848454 | Epigenetics in nucleotide repeat expansion disorders |
| Q64093623 | Erythropoietin and Friedreich Ataxia: Time for a Reappraisal? |
| Q35782974 | FXN Promoter Silencing in the Humanized Mouse Model of Friedreich Ataxia |
| Q37686297 | Fixing frataxin: 'ironing out' the metabolic defect in Friedreich's ataxia |
| Q38730963 | Friedreich Ataxia: current status and future prospects |
| Q46421445 | Friedreich ataxia-induced pluripotent stem cell-derived neurons show a cellular phenotype that is corrected by a benzamide HDAC inhibitor |
| Q38160224 | Friedreich's ataxia, frataxin, PIP5K1B: echo of a distant fracas. |
| Q38121941 | Gene regulation and epigenetics in Friedreich's ataxia |
| Q36482299 | Histone deacetylase (HDAC) inhibitors targeting HDAC3 and HDAC1 ameliorate polyglutamine-elicited phenotypes in model systems of Huntington's disease. |
| Q42068752 | Histone deacetylase-3 interacts with ataxin-7 and is altered in a spinocerebellar ataxia type 7 mouse model |
| Q38240733 | Histone deacetylases and their inhibitors in cancer, neurological diseases and immune disorders |
| Q40783140 | Improved Histone Deacetylase Inhibitors as Therapeutics for the Neurodegenerative Disease Friedreich's Ataxia: A New Synthetic Route |
| Q37155503 | Increasing frataxin gene expression with histone deacetylase inhibitors as a therapeutic approach for Friedreich's ataxia. |
| Q36026884 | Interferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model. |
| Q40533450 | Lentivirus-meditated frataxin gene delivery reverses genome instability in Friedreich ataxia patient and mouse model fibroblasts |
| Q28551371 | Lymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's Ataxia |
| Q38034809 | Management and therapy for cardiomyopathy in Friedreich's ataxia |
| Q35149824 | Mechanism of Action of 2-Aminobenzamide HDAC Inhibitors in Reversing Gene Silencing in Friedreich's Ataxia. |
| Q35893619 | Mitochondrial Diseases Part III: Therapeutic interventions in mouse models of OXPHOS deficiencies |
| Q38121943 | Mitochondrial pathophysiology in Friedreich's ataxia |
| Q33817381 | MutLα heterodimers modify the molecular phenotype of Friedreich ataxia |
| Q27022413 | New and emerging HDAC inhibitors for cancer treatment |
| Q50458074 | Open-label pilot study of interferon gamma-1b in Friedreich ataxia |
| Q28483335 | Oral administration of the pimelic diphenylamide HDAC inhibitor HDACi 4b is unsuitable for chronic inhibition of HDAC activity in the CNS in vivo |
| Q28486160 | Pharmacological screening using an FXN-EGFP cellular genomic reporter assay for the therapy of Friedreich ataxia |
| Q34488569 | Quantitative proteomic analysis identifies targets and pathways of a 2-aminobenzamide HDAC inhibitor in Friedreich's ataxia patient iPSC-derived neural stem cells. |
| Q37095593 | Rationale for the development of 2-aminobenzamide histone deacetylase inhibitors as therapeutics for Friedreich ataxia |
| Q37021501 | Reversal of epigenetic promoter silencing in Friedreich ataxia by a class I histone deacetylase inhibitor. |
| Q90477216 | SINEUP non-coding RNAs rescue defective frataxin expression and activity in a cellular model of Friedreich's Ataxia |
| Q28506707 | Silencing of Fem1cR3 gene expression in the DBA/2J mouse precedes retinal ganglion cell death and is associated with histone deacetylase activity |
| Q28087539 | The promise and perils of HDAC inhibitors in neurodegeneration |
| Q63979651 | The transcriptional regulator CCCTC-binding factor limits oxidative stress in endothelial cells |
| Q34055267 | Therapies in inborn errors of oxidative metabolism. |
| Q90522435 | Transcriptional profiling of isogenic Friedreich ataxia neurons and effect of an HDAC inhibitor on disease signatures |
| Q39230731 | Translating HDAC inhibitors in Friedreich's ataxia. |
| Q26860523 | Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular models |
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