@entity2033 ( @entity2033 ) is an autosomal dominantly @entity14827 caused by a CAG repeat expansion in the gene coding for the @entity37952 protein ( @entity25864 ). Mutant @entity25864 ( @entity25864 ) has been proposed to cause @entity528 and neuronal loss through multiple mechanisms. Transcriptional changes may be a core pathogenic feature of @entity2033 . Utilizing the Affymetrix platform we performed a genome-wide RNA expression analysis in two BACHD transgenic @entity35 lines (TG5 and TG9) at 12 months of age, both of which carry full-length @entity1 @entity25864 but with different expression levels. By defining the threshold of significance at p  <  0.01, we found 1608 genes and 871 genes differentially expressed in both TG5 and TG9 @entity35 when compared to the wild type littermates, respectively. We only chose the highly up-/down-regulated genes for further analysis by setting an additional threshold of 1.5 fold change. Comparing gene expression profiles of @entity1 @entity2033 brains and BACHD @entity35 revealed a high concordance in both functional and IPA (Ingenuity Pathway Analysis) canonical pathways relevant to @entity2033 . In addition, we investigated the causes leading to gene expression changes at molecular and protein levels in BACHD @entity35 including the involvement of @entity2035 -containing transcription factors @entity40147 ( @entity40147 ), @entity10564 and @entity7052 as well as the chromatin structure. We demonstrate that the BACHD @entity35 model recapitulates the gene expression changes of the @entity1 disease supporting its role as a preclinical research animal model. We also show for the first time that TFIID complex formation is reduced, while soluble @entity40147 is increased in an @entity2033 model. This finding suggests that @entity25864 is a competitor instead of a recruiter of @entity2035 -containing transcription factors in the transcription process in @entity2033 .

Dysregulation of gene expression in the striatum of BACHD XXXX expressing full-length mutant @entity37952 and associated abnormalities on molecular and protein levels.

@entity1 :: ('9606', 'Species') :: ['human'] @entity40147 :: ('117526(Tax:10116)', 'Gene') :: ['TATA box-binding protein', 'TBP'] @entity2035 :: ('MESH:C097188', 'Chemical') :: ['polyQ'] @entity2033 :: ('MESH:D006816', 'Disease') :: ['Huntington disease', 'HD'] @entity37952 :: ('29424(Tax:10116)', 'Gene') :: ['huntingtin'] @entity25864 :: ('15194(Tax:10090)', 'Gene') :: ['mHTT', 'HTT'] @entity10564 :: ('24790(Tax:10116)', 'Gene') :: ['Sp1'] @entity35 :: ('10116', 'Species') :: ['rat', 'rats'] @entity7052 :: ('54244(Tax:10116)', 'Gene') :: ['CBP'] @entity528 :: ('MESH:D009410', 'Disease') :: ['neuronal dysfunction'] @entity14827 :: ('MESH:D020271', 'Disease') :: ['inherited neurodegenerative disorder']
@entity35 :: (10116,Species) :: ['rats']
