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. Author manuscript; available in PMC: 2018 Apr 5.
Published in final edited form as: Neuron. 2017 Apr 5;94(1):93–107.e6. doi: 10.1016/j.neuron.2017.03.023

Figure 7. Overexpression of Ran and RanGAP1 are Neuroprotective in HD.

Figure 7

(A,B) Overexpression of HTT 82Q and eGFP in primary cortical neurons causes significant cell death compared to neurons transfected with HTT 23Q and eGFP, and cell death is significantly reduced when overexpressing RanGAP1-GFP or Ran-GFP. Experiment represents the average of 4 wells per condition. (C) Overexpression of HTT 82Q and eGFP in primary cortical neurons causes a significant reduction in cell viability compared to neurons transfected with HTT 23Q and eGFP, and is rescued when overexpressing Ran-GFP. Experiment represents the average of 4 wells total per condition over the course of two separate experiments. Data (A–C) are presented as mean ± SEM. *P<0.05, **P<0.01, ***P<0.001, and ****P<0.0001 as analyzed by One-way ANOVA followed by Tukey’s post-hoc analysis. (D) Overexpression in the Drosophila eye of HTT.Q0 or HTT.Q128 using GMR-Gal4. Co-expressing wildtype (WT) Ran rescues this disorganization, whereas co-expression of a dominant negative (DN) Ran allele enhances this disorganization phenotype. (E) Overexpression of HTT.Q128 in motor neurons using OK371-Gal4 causes lethality at the pupal stage (Fisher’s exact test, p <.0001), whereas overexpression of HTT.Q0 has no phenotype alone or with Ran alleles. Co-expression of RanWT is sufficient to partially rescue the lethality caused by HTT.Q128 expression (Fisher’s exact test, p <.0001), whereas co-expression of RanDN or GFP alone was not sufficient to rescue.