10 RESULTS
COMMENT This paper by Cook et al. describes very exciting results. The authors present strong evidence that poly(GR) promotes TDP43 pathology indirectly by inhibiting its nuclear transport and more directly through physical interaction promoting TDP43 aggregation ...
COMMENT I enjoyed reading this publication. I would be interested to know if there would be a way to restore KPNA2/4 function and/or localization and provide a phenotypic rescue downstream of DPR and TDP-43 accumulation in the cytoplasm. With regard to the ...
COMMENT It is really exciting to observe that the relocalization of FUS is independent of stress granule formation. This suggests a very unique role for TNPO transportins as well, since their relocalization to the cytoplasm is also independent of stress granule ...
COMMENT I am surprised to see data that TDP-43 export is occurring through passive diffusion rather than active export. I am not sure this will have huge implications on the effectiveness of KPT-350, as it seems to promote neuronal health independent of direct ...
COMMENT I consider all three papers to be significant contributions to our understanding of the role of DPR translation in ALS. The stimulation of DPR translation by the integrated stress response is an exciting finding, and the induction of a feed-forward ...
COMMENT I think this paper provides nice mechanistic insight into the nuclei-cytoplasmic transport defects that my paper and the Rothstein Nature paper demonstrated (Freibaum et al., 2015; Zhang et al., 2015) and further suggests that protein rather than RNA ...
COMMENT The most striking result of this complex transcriptome analysis was the specificity of C9ORF72-ALS cases to have alterations in the unfolded protein response pathway. It was reassuring to see that many of the genes with altered splicing were the same ALS ...
COMMENT Barmada et al. demonstrate that enhancing the non-sense mediated decay pathway rescues toxicity seen by the overexpression of TDP-43 and FUS. This rescue of toxicity is likely through a direct mechanism by which the auto-regulation of TDP-43 and FUS by ...
COMMENT Cooper-Knock and colleagues report the presence of increased antisense foci in motor neurons relative to sense foci. There are a few possible reasons why this may occur: 1) It is possible that there is increased transcription of antisense RNA relative to ...
COMMENT This paper by Liqun Liu-Yesucevitz and colleagues is an important contribution in understanding the role that TDP-43 plays in human diseases such as ALS and FTLD. This is a solid publication that confirms previous studies that demonstrate TDP-43 is ...
