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TranscriptomicsThe transcriptional modulator H2AFY marks Huntington's disease activity in men and mice
To accelerate the development of disease-modifying therapeutics for Huntington’s disease (HD), a dynamic biomarker of disease activity and treatment response is critically needed. Venous cellular whole blood from 8 patients with Huntington's disease (HD) and 6 control subjects were collected in PAXg...
ORGANISM(S): Homo sapiens 
2011-09-01 | E-GEOD-24250 | biostudies-arrayexpress
TranscriptomicsStudy of Huntington disease by analyzing RNA-Seq data using fly and mouse models
In this study, we perform deep mRNA sequencing to study the effect of a few gene modifiers in Huntington disease using fly models. Analysis of 3 fly RNA-Seq samples and 4 mouse RNA-Seq samples
ORGANISM(S): Mus musculus 
2014-09-05 | E-GEOD-32192 | biostudies-arrayexpress
ProteomicsIntegration-independent Transgenic Huntington Disease Fragment Mouse Models
Data from Integration-independent Transgenic Huntington Disease Fragment Mouse Models Reveal Distinct Phenotypes and Life Span in Vivo CoIP of HTT full length and fragment proteins from mouse cortical lysates. Controlled with preimmune mouse IgG IP.
ORGANISM(S): Mus Musculus (ncbitaxon:10090) 
2015-06-30 | MSV000079178 | MassIVE
TranscriptomicsDepletion of p62 reduces nuclear inclusions and paradoxically ameliorates disease phenotypes in Huntington's model mice
Huntington's disease (HD) is a dominantly inherited genetic disease caused by mutant huntingtin (htt) protein with expanded polyglutamine tracts. A neuropathological hallmark of HD is the presence of neuronal inclusions of mutant htt. p62 is an important regulatory protein in selective autophagy, ...
ORGANISM(S): Mus musculus 
2014-10-10 | E-GEOD-62210 | biostudies-arrayexpress
TranscriptomicsInhibition of transglutaminase 2 mitigates transcriptional dysregulation in models of Huntington disease
Huntington Disease (HD) is a dominantly inherited, relentlessly progressive neurodegenerative disease. Caused by a polyglutamine expansion in the mutant huntingtin protein (mhtt), HD pathogenesis impairs function in the cerebral cortex and in medium spiny neurons of the striatum and involves transc...
ORGANISM(S): Mus musculus 
2010-04-08 | E-GEOD-21237 | biostudies-arrayexpress
TranscriptomicsEuglycemic agent-mediated hypothalamic transcriptomic manipulation in the N171-82Q model of Huntington's disease is related to their physiological efficacy
Our aim was to find an effective treatment for the dysfunction in energy regulation and hypothalamic integrity occurring in many HuntingtonM-bM-^@M-^Ys disease (HD) patients and mouse models. HD is a neurodegenerative disease caused by an expanded CAG repeat and is characterized by mutant huntingtin...
ORGANISM(S): Mus musculus 
2012-10-05 | E-GEOD-39586 | biostudies-arrayexpress
TranscriptomicsMicroarray analysis of Gene expression in limb muscles of three mouse models of Kennedy disease/SBMA
Emerging evidence implicates transcriptional dyseregulation within skeletal muscle in the pathogenesis of Kennedy disease/spinal bulbar muscular atrophy (KD/SBMA). We therefore broadly characterized gene expression in skeletal muscle of three independently generated mouse models of this disorder. Th...
ORGANISM(S): Mus musculus 
2010-10-12 | E-GEOD-10190 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of mouse striatum from 12 months-old Hdh4/Q80 mice and control mice.
To test the hypotheses that mutant huntingtin protein length and wild-type huntingtin dosage have important effects on disease-related transcriptional dysfunction, we compared the changes in mRNA in seven genetic mouse models of Huntington's disease (HD) and postmortem human HD caudate. Transgenic m...
ORGANISM(S): Mus musculus 
2008-06-16 | E-GEOD-9375 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of striatum from 12 weeks-old R6/2 mice and control mice (set 2)
To test the hypotheses that mutant huntingtin protein length and wild-type huntingtin dosage have important effects on disease-related transcriptional dysfunction, we compared the changes in mRNA in seven genetic mouse models of Huntington's disease (HD) and postmortem human HD caudate. Transgenic m...
ORGANISM(S): Mus musculus 
2008-06-16 | E-GEOD-9804 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of mouse striatum from 12 weeks-old R6/2 mice and control mice (set 1)
To test the hypotheses that mutant huntingtin protein length and wild-type huntingtin dosage have important effects on disease-related transcriptional dysfunction, we compared the changes in mRNA in seven genetic mouse models of Huntington's disease (HD) and postmortem human HD caudate. Transgenic m...
ORGANISM(S): Mus musculus 
2008-06-16 | E-GEOD-9803 | biostudies-arrayexpress
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