Portrait photograph of Dr. Carleton Harrington from at the Osteopathic Hospital of Maine in Portland, Maine.https://dune.une.edu/moa_photos/1002/thumbnail.jp

Daniel Mordes (5482595)

Dennis Dickson (417904)

Francesco Limone (5482616)

Joseph Klim (5482610)

Kaitavjeet Chowdhary (5482598)

Kevin Eggan (5482607)

Leonard Petrucelli (85402)

Lindsey Goodman (5482601)

Mercedes Prudencio (437933)

Nancy Bonini (5482592)

Olli Pietilainen (5482613)

Rob Moccia (5482604)

Rosa Rademakers (85403)

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 * < 0.05, ** < 0.01). (PDF 389 kb

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

Figure S2. Gene networks in C9ORF72-ALS and sporadic ALS. (a) Protein-protein interaction network derived from differentially expressed transcripts in C9ORF72-ALS cerebellum. Those transcripts that are differentially expressed in both the frontal cortex and the cerebellum in C9ORF72-ALS are highlighted by dashed yellow circles, and predominantly consist of heat shock proteins and protein chaperones. (b) Protein-protein interaction network derived from differentially expressed transcripts in the sporadic ALS cortex. (PDF 164 kb

Additional file 6: of Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients

Figure S5. External eye quantification scale for (GR)36 animals. For quantification of the enhancement effects of increased expression of HSF in the eye, (GR)36 animals received a score between 0 (normal eye) and 11 (extreme toxicity causing lethality). Across multiple studies control Gmr-GAL4â>â(GR)36 animals receive a score between 5 and 6. (PDF 1969 kb

Additional file 3: of Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients

Osteopathic Hospital of Maine

University of New England