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TranscriptomicsFunctional genomic analysis of frataxin deficiency
This SuperSeries is composed of the following subset Series: GSE15843: Functional genomic analysis of frataxin deficiency, Agilent data GSE15848: Functional genomic analysis of frataxin deficiency, Illumina data Refer to individual Series
ORGANISM(S): Mus musculus 
2009-06-02 | E-GEOD-15849 | biostudies-arrayexpress
TranscriptomicsFunctional genomic analysis of frataxin deficiency, Illumina data
Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARγ pathway as a therapeutic target in Friedreich's ataxia Friedreich's ataxia (FRDA), the most common inherited ataxia, is characterized by focal neurodegeneration, diabetes mellitus, and l...
ORGANISM(S): Mus musculus 
2010-04-09 | E-GEOD-15848 | biostudies-arrayexpress
TranscriptomicsFunctional genomic analysis of frataxin deficiency, Agilent data
Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARγ pathway as a therapeutic target in Friedreich's ataxia Friedreich's ataxia (FRDA), the most common inherited ataxia, is characterized by focal neurodegeneration, diabetes mellitus, and li...
ORGANISM(S): Mus musculus 
2009-06-02 | E-GEOD-15843 | biostudies-arrayexpress
TranscriptomicsTranscriptome analysis of human, IPSC-derived cardiomyocytes transfected with siRNA against frataxin vs cells transfect with control siRNA.
Friedreich ataxia is a neuro and cardio degenerative disease, caused by progressive, decreased expression of the frataxin gene (FXN). Hyperthrophic cardiomyopathy is the major cause of early mortality. We used four different lines of human, Induced Pluripotent Stem Cells derived- cardiomyocytes wh...
ORGANISM(S): Homo sapiens 
2022-11-02 | E-MTAB-11296 | biostudies-arrayexpress
TranscriptomicsExpression profile of Yeast Frataxin Mutants
Yeast Frataxin Homologue 1 has been involved in oxidative stress and iron-sulfur biogenesis within the mitochondria. We have investigated the expression profile of conditional Yfh1 mutants. Yfh1 depletion leads to activation of iron uptake and repression Two strains were used: WT (strain W303-1A) an...
ORGANISM(S): Saccharomyces cerevisiae 
2013-03-06 | E-GEOD-44871 | biostudies-arrayexpress
TranscriptomicsExpression data of MCK conditional frataxin knock-out mice
Frataxin deficiency in human is the cause of Friedreich's ataxia (FA), a lethal neuro- and cardio-degenerative disease. Knock-out (KO) mice of this mouse model of FA exhibit classical cardiomyopathy of the patients. The onset of FA phenotypes in the KO mice is approximately 6-7 weeks of age. This ge...
ORGANISM(S): Mus musculus 
2011-12-31 | E-GEOD-31208 | biostudies-arrayexpress
TranscriptomicsEpigenetic therapy for Friedreich ataxia.
We set out to investigate whether a histone deacetylase inhibitor (HDACi) would be effective in an in vitro model for the neurodegenerative disease Friedreich ataxia (FRDA) and to evaluate safety and surrogate markers of efficacy in a phase I clinical trial in patients. In the neuronal cell model, H...
ORGANISM(S): Homo sapiens 
2015-01-29 | E-GEOD-65399 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of human lymphoblast cells from a Freidriechs Ataxia patient incubated with pyrrole-imidazole polyamides targeted to the GAA triplet repeat in the intron 1 reveals polyamides alleviate transcription inhibition associated with long repeat srepeats in Friedreicha??s ataxia
Lymphoblast cells from a patient with Freidriech's Ataxia were incubated with pyrrole-imidazole polyamides targeted to the GAA triplet repeat in the intron 1. The polyamides were shown in cell culture to increase levels of endogenous frataxin mRNA. A normal sibling derived lymphoblast cell line wa...
ORGANISM(S): Homo sapiens 
2008-06-13 | E-GEOD-5040 | biostudies-arrayexpress
TranscriptomicsFunctional genomic analysis of frataxin deficiency, Illumina data
Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARγ pathway as a therapeutic target in Friedreich's ataxia Friedreich's ataxia (FRDA), the most common inherited ataxia, is characterized by focal neurodegeneration, diabetes mellitus, and lif...
ORGANISM(S): Mus musculus 
2009-06-02 | GSE15848 | GEO
TranscriptomicsFunctional genomic analysis of frataxin deficiency, Agilent data
Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARγ pathway as a therapeutic target in Friedreich's ataxia Friedreich's ataxia (FRDA), the most common inherited ataxia, is characterized by focal neurodegeneration, diabetes mellitus, and lif...
ORGANISM(S): Mus musculus 
2009-06-02 | GSE15843 | GEO
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