Additional file 1: of Partial Tmem106b reduction does not correct abnormalities due to progranulin haploinsufficiency

Figure S1. Measurement of Glycosylated HexA and GCase by Western Blot. The western blots in fig. 3 measured the glycosylated forms of HexA and GCase, which was confirmed by loss of these bands after digestion with the glycosidase PNGase F. The glycosylated forms of HexA and GCase are labeled with black arrows, the unglycosylated forms with gray arrows, and nonspecific bands with asterisks. (DOCX 195 kb

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 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 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 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 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

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 1: of TMEM106B haplotypes have distinct gene expression patterns in aged brain

Table S1. Tissue samples available and selected for inclusion in this study. Table S2. DEGS in TCX. Positive fold change represents higher gene expression in SS than TT. Negative fold change represents lower gene expression in SS than TT. Table S3. DEGS in CER. Positive fold change represents higher gene expression in SS than TT. Negative fold change represents lower gene expression in SS than TT. Table S4. Overlapping genes between TCX and CER based on top 500 genes with |FC| ≥ 1.2 ranked by unadjusted p value. Table S5. Enrichment of modules for their respective DEG signatures. Table S6. Significant modules identified in the TCX and CER matched cases. Table S7. Significant modules identified in separate disease groups in TCX and CER. Table S8. Significant modules identified in the TCX and CER controls. (DOCX 54 kb

Additional file 1: Figure S1. of Slowly progressive dementia caused by MAPT R406W mutations: longitudinal report on a new kindred and systematic review

Additional neuropathology. Additional photomicrographs from three family members. Individuals, immunohistochemistry, and enlargement as indicated. (TIF 11179 kb