Table 2.
Subject characteristics of TDP-43, α-synuclein and off-target binding studies
| Case | Clinical diagnosis | Neuropathologic diagnosis | TDP type | Tau isoform | Braak | Thal | Age | Sex | Fig. |
|---|---|---|---|---|---|---|---|---|---|
| TDP-43 | |||||||||
| 6 | svPPA | AD (HpSp)/DLBD | 0 | 3R + 4R | VI | 5 | 60 | M | |
| 29 | svPPA | AD | 0 | 3R + 4R | VI | 5 | 77 | M | Fig. 7 A-C |
| 30 | agPPA | FTLD-TDP/HpScl (GRN) | A | -- | 0 | 0 | 64 | M | Fig. 7 D-F; Fig. 8 D-I |
| 31 | FTD | FTLD-TDP/HpScl (GRN) | A | -- | 0 | 0 | 63 | M | Fig. 7G-I |
| 32 | svPPA | FTLD-TDP | C | -- | 0 | 1 | 75 | M | Fig. 7 J-L |
| 33 | agPPA | FTLD-TDP | C | -- | I | 1 | 65 | M | |
| 16 | svPPA | PiD | 0 | 3R | 0 | 0 | 74 | M | |
| α-Synuclein | |||||||||
| 34 | DLB | DLBD | 0 | -- | 0 | 0 | 60 | M | |
| 35 | AD v DLB | DLBD | 0 | -- | II-III | 1 | 76 | M | |
| 36 | MSA | MSA (SND & OPCA) | 0 | -- | 0 | 1 | 58 | F | |
| Off-target | |||||||||
| 1 | Normal | Normal | 0 | -- | 0 | 0 | 81 | F | Fig. 9 A,B |
| 3 | Normal | Normal | 0 | -- | 0 | 0 | 65 | M | Fig. 9 C,D |
| 19 | PSP | PSP | 0 | 4R | II-III | 1 | 68 | F | Fig. 9 E,F |
| 6 | svPPA | AD (HpSp)/DLBD | 0 | 3R + 4R | VI | 5 | 60 | M | Fig. 9 G,H |
| 36 | MSA | MSA (SND & OPCA) | 0 | -- | 0 | 1 | 58 | F | Fig. 9 I,J |
| 30 | agPPA | FTLD-TDP/HpScl (GRN) | A | -- | 0 | 0 | 64 | M | Fig. 9 K,L |
| 2 | Normal | Normal | 0 | -- | 0 | 0 | 68 | M | Fig. 9 M-P |
| 31 | FTD | FTLD-TDP/HpScl (GRN) | A | -- | 0 | 0 | 63 | M | Fig. 9 Q-T |
| 37 | CBD | NBIA | 0 | 3R + 4R | V | 0 | 51 | M | |
| 38 | VaP | Siderosis | 0 | -- | 0 | 0 | 59 | F | |
De-identified case numbers are referred to throughout the text and within Figs. TDP-43 type was performed using the recommended harmonized classification system [33]. Braak refers to neurofibrillary tangle stage [29] and Thal refers to amyloid phase [37]. Abbreviations: svPPA semantic variant of primary progressive aphasia, agPPA agrammatic variant of PPA, FTD frontotemporal dementia, DLB dementia with Lewy bodies, AD Alzheimer’s disease, MSA multiple systems atrophy, PSP progressive supranuclear palsy, CBD corticobasal degeneration, VaP vascular parkinsonism, HpSp hippocampal sparing AD, FTLD-TDP frontotemporal lobar degeneration of a TDP-43 etiology, HpScl hippocampal sclerosis, GRN progranulin mutation, NBIA neurodegeneration with brain iron accumulation, 3R 3 repeat tau, 4R 4 repeat tau, M male, and F female