Dipeptide repeat protein inclusions are rare in the spinal cord and almost absent from motor neurons in C9ORF72 mutant amyotrophic lateral sclerosis and are unlikely to cause their degeneration

Table 2 TDP-43 and DPR scoring

	TDP-43 agg.	Poly-GP			Poly-GA			Poly-GR			Poly-PR			Poly-PA			Diagnosis
case	TDP-43 agg.	Cyt.	Nuc.	Tot.	Cyt.	Nuc.	Tot.	Cyt.	Nuc.	Tot.	Cyt.	Nuc.	Tot.	Cyt.	Nuc.	Tot.
1	3	0	0	0	0	0	0	0	0	0	0	0	0	0	0	0	ALS-TDP
2	3	0	0	0	0	0	0	0	0	0	0	0	0	0	0	0	ALS-TDP
3	2	0	0	0	0	0	0	0	0	0	0	0	0	0	0	0	ALS-TDP
4	3	0	0	0	0	0	0	0	0	0	0	0	0	0	0	0	ALS-TDP
5	3	0	0	0	0	0	0	0	0	0	0	0	0	0	0	0	ALS-TDP
MEAN ± SEM	2.8 ± 0.2																ALS-TDP
6	3	0.5	0	0.5	0	0	0	0.5	0	0.5	0	0	0	0.5	0	0.5	ALS-C9 + ve
7	3	0.5	0	0.5	1	0.5	1.5	0	0	0	0	0	0	0	0	0	ALS-C9 + ve
8	2	1	0	1	0.5	0	0.5	0.5	0	0.5	0	0	0	0.5	0	0.5	ALS-C9 + ve
9	3	1	0	1	1	0.5	1.5	0	0	0	0	0	0	0.5	0	0.5	ALS-C9 + ve
10	3	0.5	0	0.5	0.5	0.5	1	0.5	0	0.5	0	0	0	0	0	0	ALS-C9 + ve
11	3	1	0	1	1	0.5	1.5	0	0.5	0.5	0.5	0	0.5	0.5	0	0.5	ALS-C9 + ve
12	3	0.5	0	0.5	0.5	0	0.5	0.5	0	0.5	0.5	0.5	1	0	0	0	ALS-C9 + ve
13	3	1	0	1	1	0.5	1.5	0.5	0	0.5	0.5	0	0.5	0.5	0	0	ALS-C9 + ve
14	3	1	0	1	0.5	0	0.5	0	0	0	0	0	0	0	0	0	ALS-C9 + ve
15	3	0.5	0	0.5	1	0	1	0	0	0	0	0	0	0	0	0	ALS-C9 + ve
MEAN ± SEM	2.9 ± 1	0.7 ± 0.1	0.0	0.7 ± 0.1	0.7 ± 0.1	0.2 ± 0	0.9 ± 0.1	0.3 ± 0.1	0.0 ± 0.1	0.3 ± 0.1	0.1 ± 0.1	0.0 ± 0.1	0.2 ± 0.1	0.2 ± 0.1	0.0 ± 0.1	0.3 ± 0.1

Scoring the frequency of TDP-43 and DPR inclusions in the spinal cord of C9ORF72 mutation negative (1–5) and positive ALS cases (6–15). Cyt- cytoplasmic inclusion. Nuc- Nuclear inclusion. Scoring criteria: 0 – Absent 0.5 – one or two inclusions in whole section, 1- very few, 2- occasional- easy to find and a few cells are affected, 3- moderate- many of the cells are affected, inclusions are easy to find, 4- numerous- nearly all of the cells are affected

ISSN: 2051-5960