The aim of this study is to investigate tissue-specific changes in RNA expression and alternative splicing events associated with the early stages of ALS/FTD using frontal cortex (FCx), hippocampus (Hp), and spinal cord (SC) tissues isolated from mutant FUS (ΔNLS-FUS) Tg mice at an age corresponding to the early symptoms of ALS/FTD. This evidence concerns the gene FUS and frontotemporal dementia.