5 RESULTS
COMMENT I was really impressed by this work by Seddighi and colleagues for several reasons. First, this is one of the clearest demonstrations that changes at the RNA level in models of disease have substantial and consequential impacts at the protein level. Secon
COMMENT In general, this is a beautiful study of TDP43 variant dynamics in vitro and in cultured cells, focusing on the changes in cytoplasmic TDP43 mobility upon cellular stress. One of the most important findings is that HSPB1 acts as a chaperone for TDP43, reg
COMMENT This manuscript touches on a central theme in ALS, FTD, and related neurodegenerative disorders—the redistribution of RNA binding proteins in association with neurodegeneration. The trigger for cytoplasmic accumulation of these proteins, including TDP43 a
COMMENT Overall, I was very impressed by this study of Ederle et al. This is one among a series of manuscripts, including the Pinarbarsi study and our own (all published in Scientific Reports), that show convincingly that nuclear export of TDP-43 and FUS are not
COMMENT We believe that UPF1 does, in fact, have beneficial effects on neuronal survival that are independent of TDP43 and/or FUS autoregulation. This is difficult to study by removing the functional autoregulatory sequences from TDP43 or FUS because we would hav
