Gene expression profiling of R6/2 transgenic mice with different CAG repeat lengths reveals genes associated with disease onset and progression in Huntington's disease

► We compared CNS expression profiles of HD transgenic mice with 300 or 150 CAG repeats. ► We find that genes with upregulated expression distinguish these two lines. ► Lrsam1 is one gene upregulated specifically in HD mice with 300 CAGs. ► Lrsam1 prevents the loss of NeuN-positive cells and aggregates in HD striatal cultures.