Browsing by Subject C9orf72

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  • OER000000223.pdf.jpg
  • Journal article


  • Authors : Frottin, Frédéric; Pérez-Berlanga, Manuela; Hartl, F. Ulrich (2020)

  • Gain of toxic function effects have been attributed to either the DPRs or the pathological G4C2 RNA. Here we analyzed in a cellular model the relative toxicity of DPRs and RNA. Cytoplasmic poly-GA aggregates, generated in the absence of G4C2 RNA, interfered with nucleocytoplasmic protein transport, but had little effect on cell viability. In contrast, nuclear poly-GA was more toxic, impairing nucleolar protein quality control and protein biosynthesis.&#x...