Global brain transcriptome analysis of a Tpp1 Neuronal Ceroid Lipofuscinoses Mouse Model

We analyzed expression changes in the Tpp1-/- forebrain/midbrain and cerebellum of 4 mo. mutants compared to strain-related controls RNA-sequencing technology. The objective of this study was to perform, for the first time in a classical late-infantile form of Neuronal ceroid lipofuscinoses model, a transcriptome wide characterization by RNA-seq of two macro-areas mostly affected in the 4-mo.-old Tpp1-/- (cerebellum (Cb) and forebrain/midbrain (F/M)). We used a mouse model previously developed by the Lobel laboratory (J. Neurosci. 2004, 24(41)9117). Four-mo.-old males Tpp1-/- homozygous mice were compared to male wild type siblings.