Table S1 List of cases analyzed and immunohistochemistry results of Drosha staining
Genotype
Case
ALS1
ALS2
ALS3
ALS4
ALS5
ALS6
ALS7
ALS8
ALS9
ALS10
ALS11
ALS12
ALS13
ALS14
ALS15
ALS16
ALS17
ALS18
ALS19
ALS20
ALS21
ALS22
ALS23
ALS24
ALS25
ALS26
ALS27
ALS28
FTLD-TDP1
FTLD-TDP2
FTLD-TDP3
FTLD-TDP4
FTLD-TDP5
FTLD-TDP6
FTLD-TDP7
FTLD-TDP8
FTLD-TDP9
FTLD-TDP10
FTLD-TDP11
FTLD-TDP12
FTLD-TDP13
FTLD-TDP14
FTLD-TDP15
FTLD-TDP16
FTLD-TDP17
FTLD-TDP18
FTLD-TDP19
FTLD-TDP20
C1
C2
C3
C4
C5
C6
C7
C8
C9
C10
AD1
AD2
HpScl1
HpScl2
FTLD-FUS
FTLD-Tau
DLB1
DLB2
Gender
F
F
M
F
M
M
M
M
M
F
F
M
M
F
M
M
M
M
F
M
F
M
M
M
F
M
M
M
F
F
M
F
F
M
F
M
M
M
M
M
F
M
M
F
M
M
F
M
F
F
M
F
M
M
F
M
F
M
F
F
M
F
M
F
M
M
Age
62
72
82
48
59
65
63
48
77
66
72
56
74
62
71
57
39
54
56
57
69
42
60
70
59
58
54
54
63
51
48
87
72
76
62
78
99
54
69
54
54
57
73
61
77
48
76
76
74
65
55
61
68
72
65
67
68
59
84
78
62
56
51
62
73
68
TDP-43
Subtype
C
A
A
B
B
C
C
B
B
C
B
C
B
B
B
B
B
B
B
B
Presence of Drosha NCIs
GGGGCC repeats
expansion
FCx
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
-
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
+
+
NA
+
NA
+
NA
NA
NA
NA
++
++
+
NA
NA
NA
+
+
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
Hp
Cb
DG
NA
+
+
+
++
++
+
++
+
++
+
++
+
+
+
+++
NA
+
++
++
+
++
NA
+
NA
+
+
NA
-
Gr layer
NA
NA
NA
NA
NA
NA
NA
++
NA
NA
++
NA
NA
NA
NA
NA
NA
++
++
NA
NA
+
+
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
NA
Cases: ALS (Amyotrophic Lateral Sclerosis), FTLD-TDP (Frontotemporal Lobar Degeneration with TDP-43 pathology), C (control), AD (Alzheimer Disease), HpScl (Hippocampal
Sclerosis), DLB (Dementia with Lewy Bodies)
Gender: F (Female), M (Male)
Genotype: - (non GGGGCC expanded repeats), + (GGGGCC expanded repeats)
Presence of Drosha NCIs (neuronal cytoplasmic inclusions): FCx (Frontal Cortex), Hp (Hippocampus), DG (Dentate Gyrus), Cb (Cerebellum), Gr Layer (Granular layer), + (1-3
inclusions/field), ++ (3-10 inclusions/field), +++ (more than 10 inclusions/field)