Regional distribution of anchorless prion protein, PrP226*, in the human brain

Figures & data

Figure 1. Average D/N ratios for all samples. (A) D/N ratios for sCJD samples. C (black); FL (dark-gray); OL (gray); OB (light-gray); SC (white). (B) D/N ratios for non-CJD samples. Dark-gray columns represent NC (13–19) and light-gray columns represent NNC (20–30). The data was obtained from PrP226* assay. Sequential numbers of samples are as in Table 1. C, cerebellum; FL, frontal lobe; OL, occipital lobe; OB, olfactory bulb; SC, spinal cord.

Figure 2. (A) Percentage of samples from certain brain region of 12 sCJD cases, which were defined as positive. (B) Results of PrP226* assay for all sCJD cases. Sequential numbers of samples are as in

Figure 3. Denaturation profiles. D/N ratios are ploted as the function of the GdnSCN concentration. Sequential numbers of samples are as in Table 1. C, cerebellum; FL, frontal lobe; SC, spinal cord.

Figure 4. Comparison of A₄₅₀ values of non-denatured samples (all brain regions included). The first bar represents the average value of non-denatured non-CJD samples (NC, NNC). sCJD samples were divided in two groups – samples, that were defined as negative (D/n < 1.2) were included in group 1, samples, that were defined as positive (D/n > 1) were included in group 2. The second bar represents the average value of non-denatured sCJD samples from group 1 and the third bar represents the average value of non-denatured sCJD samples from group 2.

Figure 5. Influence of protease inhibitors in homogenization buffer on the amount of PrP226*. Samples were homogenized in buffer with (+) or without (-) of protease inhibitors. A₄₅₀ values are shown for non-denatured (A) and denatured (B) samples.

Table 1. Summary of data on patients included in the study

		Age	Genger	Genotype	Pathological diagnosis	Additional pathological diagnosis	Duration of disease (months)	PrPSc deposition type regarding IHC	Protein 14–3-3	Ataxia	Cerebellar signs	Visual impairment
1	case	75	F	MM	sCJD	AD	1	synaptic	ND	yes	yes	yes
2	case	72	F	MM	sCJD	AD	14	synaptic	-	ND	no	no
3	case	69	F	MM	sCJD	AD	4	synaptic	-	no	yes	yes
4	case	62	M	MM	sCJD	none	3	mixed*	+	no	yes	yes
5	case	71	F	MM	sCJD	AD + PD	18	mixed*	-	no	no	no
6	case	77	M	MM	sCJD	tauopathy	2	synaptic	+	no	no	no
7	case	70	M	VV	sCJD	AD	7	mixed*	ND	yes	yes	ND
8	case	74	F	MM	sCJD	none	5	mixed*	+	no	no	no
9	case	65	M	MM	sCJD	none	2	synaptic	+	yes	yes	no
10	case	81	F	MM	sCJD	AD	3	mixed*	+	yes	yes	yes
11	case	70	M	MM	sCJD	tauopathy	5	synaptic	+	yes	yes	ND
12	case	74	F	MV	sCJD	AD	3	mixed*	+	ND	ND	ND
13	NC	75	F		tauopathy
14	NC	68	F		AD II-III, diffuse gliosis
15	NC	69	M		AD III, multiple microinfarcts in cerebral cortex
16	NC	81	F		AD II, Binswanger disease
17	NC	60	M		AD VI subependymoma in the third ventricle
18	NC	68	M		vascular dementia
19	NC	71	F		AD III, hypoxic encephalopathy
20	NNC	84	F
21	NNC	91	F
22	NNC	77	F
23	NNC	74	F
24	NNC	74	M
25	NNC	81	F
26^§	NNC	51	F		malignant cerebral edema, subarachnoid hemorrhage
27^§	NNC	64	M		cerebral edema, subarachnoid hemorrhage
28^§	NNC	55	F		central nervous system metastases
29	NNC	69	M
30	NNC	44	M

Abbreviations: sCJD, sporadic CJD; NC, neurological control; NNC, non-neurological control; F, female; M, male; AD, Alzheimer's disease; PD, Parkinson's disease, IHC, immunohistochemistry; ND, no data; -, negative; +, positive. *More than one PrP^Sc-deposition type was determined by IHC. §These samples were included into the group of non-neurological controls despite the neurological condition, due to the absence of neurodegenerative disease. In all other non-neurological controls, nervous system was not impaired.