SCA17 knock-in mice brain transcriptomes

To identify brain region specific proteins that contribute to brain region specific neurodegeneration, RNA sequencing was performed using cerebellum, striatum and prefrontal cortex tissues from 3-month-old SCA17 knock-in (KI) mice and wild-type (WT) littermates. We find 245 cerebellar specifically dysregulated genes (110 up-regulated genes and 145 down-regulated genes), 133 striatum specifically dysregulated genes (45 up-regulated genes and 88 down-regulated genes) and 17 prefrontal cortex specifically dysregulated genes (4 up-regulated genes and 13 down-regulated genes). Combined with other evidences, we demonstrate that cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of Spinocerebellar ataxias type 17 To compare RNA level of cerebellum, striatum and prefrontal cortex from 3-month old WT and KI mice of SCA-17 by RNA sequencing, each group consists three mice.