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Table 2 Ratings for p62 and DPR antibody immunostaining of neuronal cytoplasmic inclusions for all 15 cases with expansions in C9ORF72

From: Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72

Case CA4 DG GCC
  p62 poly-GA poly-GP poly-GR poly-PR poly-AP p62 poly-GA poly-GP poly-GR poly-PR poly-AP p62 poly-GA poly-GP poly-GR poly-PR poly-AP
1* 2 2 3 3 0.5 0.5 1 2 0 0.5 0.5 0.5 4 4 3 0.5 0 0
2* 3 3 2 3 0.5 1 2 2 0.5 2 0.5 1 2 3 3 0 0 0
3* 3 3 3 2 0.5 0.5 4 3 3 0 0 0 1 4 2 2 0 0
4* 2 2 3 3 0.5 0 3 2 3 0 0.5 0 3 3 3 0 0 0
5* 2 1 3 3 0.5 0.5 3 3 2 0 0 0 2 3 3 1 0 0
6* 1 2 2 1 0 0 1 2 2 0 0 0 1 1 1 0 0 0
7** 3 3 3 3 0 0 3 3 2 0 0 0 4 4 4 0 0 0
8** na na na na na na na na na na na na 1 1 1 0 0 0
9** 3 2 3 3 0.5 0.5 1 2 1 0.5 0 0 0.5 3 1 0 0 0
10** 3 2 2 2 0.5 0.5 4 3 3 1 0.5 0 3 3 2 0 0 0
11** 3 3 3 3 0 0 4 3 2 2 0 1 0.5 3 2 1 0.5 0
12** 3 3 2 3 0 0 4 3 1 1 0 0 1 3 3 1 1 0
13 0 2 2 2 0 0 0 2 2 0 0 0 1 1 1 0 0 0
14 3 2 3 3 0 1 1 2 2 0 0 0 3 4 2 0 0 0
15 1 1 1 1 0 0 2 2 2 2 0 0 1 1 1 0 0 0
16 2 2 2 0 0 0 2 2 2 2 0 0 3 3 3 0 0 0
  1. Cases #1-6 had FTLD TDP-43 type A histology (*), cases #7-12 had FTLD TDP-43 type B histology (**), case #13 had corticobasal degeneration, and cases #14-16 had Motor Neurone Disease.
  2. CA4 = pyramidal cells of CA4 region of hippocampus; DG = dentate gyrus granule cells; GCC = granule cells of the cerebellum; na = no data.