Additional file 9: of Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients

Figure S4. poly-GR expression results in the upregulation of heat shock response genes and dHSF1 in the adult fly nervous system. UAS-(GR)36 was expressed in the adult fly nervous system using the drug-inducible Gal4 driver, elavGS, for 16d. qPCR analysis of endogenous HSF1-regulated genes and dHSF1 revealed significant upregulation of the Drosophila orthologs of many of the genes identified in patient studies, suggesting that poly-GR is contributing to the altered transcriptome in C9ORF72-ALS/FTLD patients. Control animals did not express a transgene. Differences in expression are likely underestimated as the analyses include neuronal and non-neuronal tissue while (GR)36 was expressed only in neurons. (n = 6, mean +/− SD, unpaired, student’s t-tests, p-value *