Activation of the unfolded protein response and granulovacuolar degeneration are not common features of human prion pathology

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

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