8 research outputs found
Carleton Harrington D.O.
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
Additional file 8: of Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients
Figure S3. Activation of HSF1 in C9ORF72-ALS, FTLD, and combined ALS/FTLD patients. (a) Quantitative real-time PCR (qRT-PCR) for HSF1 target genes in the cerebellum of sporadic and C9ORF72-associated disease (n = 56 C9ORF72-ALS/FTLD, n = 42 sporadic ALS/FTLD, n = 7 controls) (one-way ANOVA with Bonferonni post-hoc test for multiple comparisons * p < 0.05, ** p < 0.01, *** p < 0.001. Note, no significant changes were detected between the sporadic cases and controls. (b) Correlation of HSF1 levels and HSF1 target gene levels in the frontal cortex and cerebellum in C9ORF72-ALS/FTLD. Spearman’s R2 values are plotted for each target gene, error bars denote 95% confidence interval, p-value < 0.0001 in all cases. (PDF 209 kb
Additional file 7: of Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients
Table S4. Values from qPCR analysis in brain samples. (PDF 178 kb
Additional file 5: of Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients
Table S3. Genes significantly changes in RNA-seq analysis from ALS patients. (XLSX 52 kb
Additional file 4: of Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients
Figure S1. Bioinformatic method comparison for gene expression analysis of C9ORF72-associated ALS and sporadic ALS (sALS) in the frontal cortex and cerebellum. (a-d) Gene density plots comparing the expression levels of differentially expressed transcripts as determined by the prior double cut-off method (|log2 fold change| ≥ 2 and p-value < 0.05) and the FDR method (FDR < 0.05), which was used in Fig. 1 and subsequent analysis. Note, no significant changes were detected in the sALS cerebellum using with FDR method. (e) Venn diagrams demonstrating considerable differences in the designated disease-associated transcripts between these bioinformatics methods in both brain regions. (f) Table of the number of differentially expressed transcripts as determined by Prudencio et al., the double cut-off method and the FDR method used here. (PDF 216 kb
Additional file 3: 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-GAL4 > (GR)36 animals receive a score between 5 and 6. (PDF 1969 kb
Additional file 6: 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 2: of Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients
Table S2. Primer sequences for PCR. (PDF 167 kb