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. Author manuscript; available in PMC: 2019 Jul 1.
Published in final edited form as: Trends Neurosci. 2018 May 2;41(7):457–469. doi: 10.1016/j.tins.2018.04.002

Figure 3. Overview of the Nucleocytoplasmic Transport Defects in C9ORF72-ALS/FTD.

Figure 3

This cartoon summarizes the identified nuclear cytoplasmic transport defects in Drosophila, yeast and C9ORF72 iPSC-derived neurons. G4C2 quadruplex RNA disrupts the RAN gradient and the nuclear import of proteins by binding to RanGAP, which catalyzes the conversion of Ran-GTP to Ran-GDP. Impairment of nuclear export caused by repeat RNA or DPR proteins leads to nuclear retention of RNA.