Comparative gene expression profiling analysis of small RNA-seq data in FUS-D14 mutant, FUS KO and SETX-R2136H mutant adult mouse spinal cords compared to respective littermate WT controls
There is growing evidence that there is dysfunction in the processing of small RNA in sporadic and familial ALS more generally. The dysfunction in miRNA processing in human patient tissue and ALS models have been described to have various downstream toxic effects, and therefore may be an important part of ALS pathology. Mutations in FUS are causative for early-onset ALS. We compared a FUS mutant mouse model, FUS D14, and a FUS-KO model to understand the role of gain and loss of function in FUS D14 on miRNA dysregulation. We also compared this to another ALS model, SETX-R2136H.