. Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA. Science. 2016 Jan 8;351(6269):173-6. Epub 2015 Dec 3 PubMed.


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  1. 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 toxicity might be the more important factor in C9orf72-mediated neurotoxicity. The mislocalization of THOC2 and the suggestion of RNA processing defects in disease is consistent with our identification of THOC4(ALYREF), the nuclear cap binding complex (NCBP1, NCBP2, ARS2), and exosomal subunits as genetic interactors of C9orf72 mediated toxicity.


    . GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport. Nature. 2015 Sep 3;525(7567):129-33. Epub 2015 Aug 26 PubMed.

    . The C9orf72 repeat expansion disrupts nucleocytoplasmic transport. Nature. 2015 Sep 3;525(7567):56-61. Epub 2015 Aug 26 PubMed.

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This paper appears in the following:


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