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GenomicsMutant Huntingtin promotes neuronal death through cell autonomous microglial activation via myeloid lineage- determining factors
Huntington's Disease (HD) is a fatal neurodegenerative disorder caused by an extended polyglutamine repeat in the N-terminus of the huntingtin (Htt) protein. Reactive microglia and elevated cytokine levels are observed in the brains of HD patients, but the extent to which neuroinflammation results f...
ORGANISM(S): Mus musculus 
2014-03-02 | E-GEOD-54443 | biostudies-arrayexpress
TranscriptomicsElucidating a normal function of huntingtin by analysis of huntingtin-null mouse embryonic fibroblasts
The polyglutamine expansion in huntingtin (Htt) protein is a cause of Huntington’s disease (HD). Htt is an essential gene as deletion of the mouse Htt gene homolog (Hdh) is embryonic lethal in mice. Therefore, in addition to elucidating the mechanisms responsible for polyQ-mediated pathology, it i...
ORGANISM(S): Mus musculus 
2008-04-12 | E-GEOD-11139 | biostudies-arrayexpress
TranscriptomicsDiscovery of novel isoforms of Huntingtin reveals a new hominid-specific exon
Huntington’s disease (HD) is a devastating neurological disorder that is caused by an expansion of the poly-Q tract in exon 1 of the Huntingtin gene (HTT). HTT is an evolutionarily conserved and ubiquitously expressed protein that has been linked to a variety of functions including transcriptional r...
ORGANISM(S): Homo sapiens 
2015-06-10 | E-GEOD-66769 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of striatal samples from 4 CHL2 Q150/Q150 mutant mice and 4 age-matched wild-type mice to investigate the study of huntingtin toxicity and its remedy in model systems.
Achieving a mechanistic understanding of disease and initiating preclinical therapeutic trials necessitate the study of huntingtin toxicity and its remedy in model systems. To allow the engagement of appropriate experimental paradigms, Huntington’s disease (HD) models need to be validated in terms...
ORGANISM(S): Mus musculus 
2008-04-10 | E-GEOD-10202 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of striata from PGC-1a knockout mice and litter mate controls to understand the roles of PGC-1a.
Huntington's Disease (HD) is an inherited neurodegenerative disease caused by a glutamine repeat expansion in huntingtin protein. Transcriptional deregulation and altered energy metabolism have been implicated in HD pathogenesis. We report here that mutant huntingtin causes disruption of mitochondr...
ORGANISM(S): Mus musculus 
2007-11-13 | E-GEOD-5786 | biostudies-arrayexpress
GenomicsExtensive changes in DNA methylation are associated with expression of mutant huntingtin [RRBS]
The earliest stages of Huntington’s disease are marked by changes in gene expression that are caused in an indirect and poorly understood manner by polyglutamine expansions in the huntingtin protein (HTT). To explore the hypothesis DNA methylation may be altered in cells expressing mutated HTT, we ...
ORGANISM(S): Mus musculus 
2013-01-24 | E-GEOD-43432 | biostudies-arrayexpress
TranscriptomicsExtensive changes in DNA methylation are associated with expression of mutant huntingtin [mRNA-seq]
The earliest stages of Huntington’s disease are marked by changes in gene expression that are caused in an indirect and poorly understood manner by polyglutamine expansions in the huntingtin protein (HTT). To explore the hypothesis DNA methylation may be altered in cells expressing mutated HTT, we ...
ORGANISM(S): Mus musculus 
2013-01-24 | E-GEOD-43431 | biostudies-arrayexpress
GenomicsExtensive changes in DNA methylation are associated with expression of mutant huntingtin [MeDIP-seq]
The earliest stages of Huntington’s disease are marked by changes in gene expression that are caused in an indirect and poorly understood manner by polyglutamine expansions in the huntingtin protein (HTT). To explore the hypothesis DNA methylation may be altered in cells expressing mutated HTT, we ...
ORGANISM(S): Mus musculus 
2013-01-24 | E-GEOD-43430 | biostudies-arrayexpress
GenomicsExtensive changes in DNA methylation are associated with expression of mutant huntingtin [ChIP-seq]
The earliest stages of HuntingtonM-bM-^@M-^Ys disease are marked by changes in gene expression that are caused in an indirect and poorly understood manner by polyglutamine expansions in the huntingtin protein (HTT). To explore the hypothesis DNA methylation may be altered in cells expressing mutate...
ORGANISM(S): Mus musculus 
2013-01-24 | E-GEOD-43429 | biostudies-arrayexpress
ProteomicsPeptide mapping of purified huntingtin protein samples from different eukaryotic expression systems
Huntingtin expression, purification and biophysical characterisation: purified huntingtin protein samples of different polyQ lengths derived from insect cell and mammalian cell production were digested and subject to peptide mass spectrometry.
ORGANISM(S): Spodoptera frugiperda Homo sapiens (Human) 
2019-03-12 | PXD010865 | Pride
Pbacmam Post-translational modification Huntingtin Huntington’s disease Polyglutamine Eukaryotic expression system Htt Hd Polyq 
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