Brain transcriptome analysis of a TPP-1 neuronal ceroid lipofuscinosis mouse model as disease progresses

We analyzed expression changes in the Tpp1-/- forebrain/midbrain and cerebellum of 1, 2, and 3 mo. mutants compared to strain-related controls RNA-sequencing technology. Overall design: The objective of this study was to perform a transcriptome wide characterization by RNA-seq of two affected brain-areas (cerebellum (Cb) and forebrain/midbrain (F/M)) at 1-, 2- and 3-mo.-old Tpp1-/- compared to age matched wild type siblings. We used a mouse model previously developed by the Lobel laboratory (J. Neurosci. 2004, 24(41)9117).