Gene expression and behaviour in mouse models of HD

Huntington's disease (HD) is an autosomal dominant neurodegenerative disease, resulting in expansion of the CAG repeat in exon 1 of the HTT gene. The resulting mutant huntingtin protein has been implicated in the disruption of a variety of cellular functions, including transcription. Mouse models of HD have been central to the development of our understanding of gene expression changes in this disease, and are now beginning to elucidate the relationship between gene expression and behaviour. Here, we review current mouse models of HD and their characterisation in terms of gene expression. In addition, we look at how this can inform behaviours observed in mouse models of disease. The relationship between gene expression and behaviour in mouse models of HD is important, as this will further our knowledge of disease progression and its underlying molecular events, highlight new treatment targets, and potentially provide new biomarkers for therapeutic trials.

► We review the current mouse models of Huntington's Disease.
► We examine the evidence for the relationship between gene expression and behaviour in mouse models generally, and in HD mouse models specifically.
► Discrepancies between mRNA and protein levels may be a result of technical noise, plus the effect of post-translational modifications.
► Epigenetic s is likely to be important in explaining the mechanism relating gene expression and behaviour.
► Investigating the relationship between gene expression and behaviour may inform understanding of disease progression and new therapeutics.