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. 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).

本研究采用RNA测序（RNA-seq）技术，对比分析1、2、3月龄Tpp1⁻/⁻突变小鼠的前脑/中脑与小脑的基因表达变化，对照样本为同品系小鼠。本研究的核心目标为：通过RNA-seq技术，对1、2、3月龄Tpp1⁻/⁻小鼠的两个受累脑区——小脑（Cb）与前脑/中脑（F/M）——开展全转录组特征分析，对照样本为年龄匹配的野生型同窝小鼠。本研究使用的小鼠模型由Lobel实验室此前构建，相关研究发表于《Journal of Neuroscience》2004年第24卷第41期，第9117页。