Polyglutamine expansion in TBP alters transcription in cellular model of SCA17

The poorly conserved N-terminal half of TATA binding protein (TBP) harbours a polyglutamine stretch of 29 to 42 in normal individuals. Expansion of this stretch leading to more than 42 is associated with a neurodegenerative disorder spinocerebellar ataxia 17 (SCA17). This study compares the espression profile of mouse neuro 2a cells expressing a vector control and TBP varient with 59 polyglutamines known to form intra nuclear aggregates. Keywords: Neuro 2a, SCA17, TBP, polyglutamine Mouse Neuro 2a cells transfected with expression vector and clones expressing TBP varients having stretch of 59 glutamines were compared by cDNA microarray analysis. The study includes four biological replicates and two pairs of flip dye replicates. RNA was extracted and labelled with TSA labeling system (Perkin-Elmer Life Sciences, USA) before hybridization.