Amyotrophic Lateral Sclerosis associated FUS mutation shortens mitochondria and induces neurotoxicity

We employ CLIP-Seq, RNA-Seq and Ribo-Seq in cultured neurons expressing R495X or wild-type FUS to identify effects of the mutation on the FUS targetome and on the neuronal transcriptome at the expression and translation level We set up an inducible expression system based on lentiviral transduction of FLAG-tagged human R495X or wild-type FUS in neurons differentiated from mouse Embryonic Stem cells. we performed CLIP-Seq , RNA-Seq and Ribo-Seq in neurons expressing WT or R495X.