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Table 1 Characterization and quantification of pTDP-43-immunopositive spinal cord motor neuron (MN) inclusions, as well as diffuse cytoplasmic pTDP-43 staining and nuclear pan TDP-43 immunoreactivity, in seven micron paraffin-embedded formalin-fixed tissue sections of the cervical and thoracic spinal cord from all post-mortem tissue cases

From: Co-deposition of SOD1, TDP-43 and p62 proteinopathies in ALS: evidence for multifaceted pathways underlying neurodegeneration

Case #

Diagnostic group

pTDP-43 morphology

Quantification (%MNs)

Globular

Skein-like

Punctate

Diffuse

Incl./diff

Nuclear

1

Age-matched control

+++

2

Age-matched control

NA

NA

NA

NA

NA

NA

3

Age-matched control

+++

4

Age-matched control

+

++

5

Age-matched control

+++

6

Age-matched control

+

+++

7

Age-matched control

+++

8

Age-matched control

++

9

Age-matched control

++

10

Age-matched control

+

++

11

fALS (SOD1, I113T)

+

+

12

fALS (SOD1, I113T)

++

+

13

fALS (SOD1, D101G)

+

+

14

fALS (C9ORF72, 30+positive)

++

+

15

fALS (unknown mutation)

++

+

16

fALS (C9ORF72, 30+positive)

++++

+

17

fALS (C9ORF72, 30+positive)

+++

+

18

sALS

+++

+

19

sALS

++++

+

20

sALS

++++

+

21

sALS

++

+

22

sALS

NA

NA

NA

NA

NA

NA

23

sALS

+++

+

24

sALS

++++

+

25

sALS

++

+

26

sALS

NA

NA

NA

NA

NA

NA

  1. Fixed tissues were not available (NA) for some cases. The four morphologies of pTDP-43 pathology were noted as present (√) or absent (−), and the proportion of motor neurons exhibiting any of the four morphologies quantified. The proportion of motor neurons exhibiting nuclear pan TDP-43 immunostaining was also quantified. Quantification classifications;− = 0%, + =  > 0–25%, ++  = 26–50%, +++ = 51–75%, ++++ = 76–100%