Additional file 4: of Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease

Figure S3. Dot plot graph of semiquantitative assessment of neurodegeneration and DPR. Note that X axis is neurodegeneration score (0 to 3), Y-axis is density of DPR. FCtx - frontal cortex, DF - dentate fascia, CA4 - cornu ammonis sector 4. (TIF 1325 kb

Additional file 3: of Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease

Table S1. Quantitative assessment of DPR density by color deconvolution algorithm in clinicopathologic subgroups of C9ORF72-related disease. In frontal cortex, p = 0.019, FTLD vs. FTLD-MND. In CA4, p = 0.055, FTLD vs. FTLD-MND. In CA2/3, p = 0.03, FTLD vs. FTLD-MND. Significant p-values (< 0.05) are indicated in bold. All variables were analyzed with Kruskal-Wallis ANOVA on Ranks and data are displayed as median (25th and 75th range). *Statistically significant p-value (p < 0.05); all p-value for ANOVA on Ranks comparison of all three groups. FCtx = frontal cortex, MCtx = Motor cortex, DF dentate fascia, CA hippocampal subfields. (DOCX 15 kb

Additional file 7: of Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease

Figure S5 Comparison of immunostaining with aDMA between C9ORF72 cases and non- neurodegeneration control in parahippocampal cortex. The nuclear signal of aDMA is variable in both cases and controls. Note sparse cytoplasmic inclusions labeled with aDMA in in C9ORF72 cases (arrows). Scale bar represents 50 ΟM. (TIF 2898 kb

Additional file 6: of Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease

Table S2. Correlation between DPR and aDMA inclusions. P-values are from Pearson’s test of correlation. Significant p-values (< 0.05) are indicated in bold. FCtx frontal cortex, DF dentate fascia, CA - cornu ammonis, MCtx motor cortex. (DOCX 14 kb

Additional file 2: of Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease

Figure S2. Correlation between manual counts and positive pixel burden from color deconvolution in poly-GR staining. Plot shows the correlation of manual counts of neuronal cytoplasmic inclusions and positive pixel burden from color deconvolution in poly-GR staining. The line shows linear regression CD color deconvolution. Ctx frontal cortex, DF dentate fascia, CA - cornu ammonis, MCtx motor cortex. (TIF 2581 kb

Additional file 5: of Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease

Figure S4. Western blot analysis of aDMA and sDMA in brains of C9FLTD-MND and sporadic FTLD-MND. The high molecular weight aDMA and sDMA signals are visible in c9FTD/ALS, but not in sporadic FTD/ALS cases. (TIF 2960 kb