Table 3.
Overview of the results obtained from immunohistochemical stainings for pIRE1α, pPERK, CK1δ, Aβ and phosphorylated tau
| Case | Neuropathological diagnosis | pIRE1α | pPERK | CK1δ | Aβ deposits | pTau (small inclusions/neuritic changes) | pTau (tangle-like) |
|---|---|---|---|---|---|---|---|
| 1 | Ctrl | 0 | 0 | 0 | - | - | - |
| 2 | Ctrl | 0 | 0 | 0 | - | - | - |
| 3 | Ctrl | 0 | 0 | 0 | - | −/+ | - |
| 4 | Ctrl | 0 | 0 | 0 | - | - | - |
| 5 | Ctrl | 0 | 0 | 0 | - | - | - |
| 6 | Ctrl | 0 | 0 | 0 | ++ | −/+ | - |
| 7 | Ctrl | 0 | 0 | 0 | - | - | - |
| 8 | Ctrl | 0 | 0 | 0 | - | −/+ | - |
| 9 | Ctrl | 0 | 0 | 0 | + | - | - |
| 10 | GSS | 0 | 0 | 2 | + | +++ | - |
| 11 | GSS | 0 | 0 | 3 | ++ | ++/+++ | - |
| 12 | GSS | 0 | 0 | 0 | - | −/+ | - |
| 13 | GSS | 0 | 0 | 0 | - | - | - |
| 14 | GSS | 0 | 10 | 21 | - | +++ | +++ |
| 15 | PrP-CAA | 1 | 0 | 0 | - | ++ | - |
| 16 | FFI | 0 | 0 | 2 | - | −/+ | - |
| 17 | FFI | 2 | 0 | 1 | ++ | ++/+++ | - |
| 18 | FFI | 0 | 0 | 2 | - | −/+ | - |
| 19 | vCJD | 0 | 0 | 0 | - | +++ | - |
| 20 | vCJD | 1 | 0 | 4 | - | +++ | - |
| 21 | vCJD | 0 | 0 | 2 | - | +++ | - |
| 22 | iCJD | 0 | 0 | 0 | - | −/+ | - |
| 23 | iCJD | 0 | 0 | 0 | - | ++ | - |
| 24 | iCJD | 0 | 0 | 1 | + | ++ | - |
| 25 | sCJD | 0 | 0 | 0 | - | + | - |
| 26 | sCJD | 0 | 1 | 0 | +++ | ++/+++ | ++ |
| 27 | sCJD | 0 | 0 | 0 | - | ++ | - |
| 28 | sCJD | 0 | 0 | 0 | + | ++ | - |
| 29 | sCJD | 0 | 0 | 0 | - | ++/+++ | - |
| 30 | sCJD | 0 | 0 | 1 | - | ++ | - |
| 31 | sCJD | 0 | 0 | 1 | - | ++ | - |
| 32 | sCJD | 1 | 0 | 0 | - | + | - |
| 33 | sCJD | 0 | 0 | 1 | +++ | + | + |
| 34 | sCJD | 0 | 0 | 2 | - | + | - |
| 35 | sCJD | 0 | 0 | 2 | - | + | - |
| 36 | sCJD | 0 | 0 | 6 | ++ | ++/+++ | ++/+++ |
| 37 | sCJD | 0 | 0 | 2 | ++ | ++ | - |
| 38 | sCJD | 1 | 0 | 1 | ++ | ++ | - |
| 39 | sCJD | 0 | 0 | 0 | + | ++ | - |
| 40 | sCJD | 1 | 0 | 0 | + | + | - |
| 41 | sCJD | 0 | 0 | 2 | - | −/+ | - |
| 42 | sCJD | 1 | 0 | 0 | ++ | ++ | - |
| 43 | sCJD | 0 | 0 | 0 | - | −/+ | - |
| 44 | sCJD | 4 | 0 | 2 | +++ | ++ | - |
| 45 | sCJD | 0 | 0 | 0 | - | −/+ | - |
| 46 | sCJD | 0 | 0 | 1 | - | ++/+++ | - |
| 47 | sCJD | 0 | 0 | 0 | - | −/+ | - |
| 48 | sCJD | 0 | 0 | 0 | - | −/+ | - |
| 49 | sCJD | 0 | 0 | 0 | ++ | −/+ | −/+ |
| 50 | sCJD | 0 | 0 | 0 | - | + | - |
| 51 | sCJD | 0 | 0 | 1 | ++ | −/+ | - |
| 52 | sCJD | 0 | 0 | 1 | - | −/+ | - |
| 53 | sCJD | 0 | 0 | 0 | + | + | - |
| 54 | sCJD (p.enceph.) | 0 | 0 | 0 | - | ++ | - |
| 55 | sCJD (p.enceph.) | 0 | 0 | 0 | - | −/+ | - |
| 56 | VPSPr | 0 | 0 | 0 | - | + | - |
| 57 | Sporadic AD | 0 | 0 | 5 | ++ | ++ | + |
| 58 | Sporadic AD | 0 | 2 | 1 | ++ | ++ | + |
| 59 | Familial AD | 14 | 83 | 204 | +++ | +++ | +++ |
pIRE1α, pPERK and CK1δ scores were obtained by quantification of the amount of positive neurons. The values are corrected for the surface area of each section. The value represents the amount of positive neurons per cm2. All values are rounded to the nearest whole number. Aβ and tau scores were semi-quantitatively assessed. See Additional file 1: Figure S1 for representative examples of the +, ++ and +++ scores of all three classes. Positive controls from AD hippocampus are not depicted in this table
Abbreviations: pTau phosphorylated tau, p. enceph. panencephalopathic subtype