Filamentous white matter prion protein deposition is a distinctive feature of multiple inherited prion diseases

Reiniger, Lilla; Mirabile, Ilaria; Lukic, Ana; Wadsworth, Jonathan DF; Linehan, Jacqueline M; Groves, Michael; Lowe, Jessica; Druyeh, Ronald; Rudge, Peter; Collinge, John; Mead, Simon; Brandner, Sebastian

doi:10.1186/2051-5960-1-8

Acta Neuropathologica Communications

Table 2 List of all cases analysed for white matter PrP in this study

From: Filamentous white matter prion protein deposition is a distinctive feature of multiple inherited prion diseases

Figure	Case no	Mutation	Codon 129 status	PrP ^Scmolecular strain type*	Gender	Age at death	Disease duration (mo)	Amount of PrP ^Scin WM		PrP pattern WM T = Threads D = Dots		Other pathology
								FB	Cer	FB	Cer	Cortical PrP pattern	Spongiosis
	1	V210L	MV	2	M	69	5	+	—	T, D	—	S1, PN1, D2	+
	2	E200K	MM	2	M	65	4	+	—	D	—	S2, GR1, PN1	+
	3	E200K	MV	ND	M	67	24	+	—	D	—	S1	+
	4	E200K	MM	ND	F	57	6	++	+	D	D	S1, PN1	+
Figure 2D-F, Figure 4A, B	5	E200K	MV	2/3	F	45	38	+++	+	T, D	D	S3, GR2	+++
	6	E200K	MM	ND	M	61	6	++	+	D	D	S2, GR2, PV2	+
	7	E200K	MM	2	M	65	5	—	—	—	—	S1, PN1	++
	8	E200K	MM	2	M	65	9	+	—	D		S1	+
	9	E200K	MM	2	F	69	4	+	+	D	—	S2, PN1	++
	10	E200K	MM	ND	M	47	19	++	++	T, D	T, D	S2, D1, PV2	+
	11	D178N	MM	Negative	M	60	7	+++	+	D	D	P3, D2	o
Figure 2A-C, Figure 3I, J	12	D178N	MV	ND	M	60	24	++	—	D	—	S2, GR1, P1	+++
	13	D178N	MV	ND	F	51	20	—	—	—	—	S1, GR1	++
	14	A117V	MV	Negative	M	43	6	+++	++	T	D	S2, P3, D3	o
	15	A117V	VV	Negative	M	40	46	+++	++	T	D	S2, P3	++
Figure 1M-P, Figure 3G, H	16	A117V	MV	Negative	M	47	47	+++	—	T	—	S1, P3	o
Figure 5C, D	17	A117V	MV	ND	F	40	98	+++	+	T	T, D	S2, P3	++
	18	P102L	MV	Negative	M	53	64	++	++	T	T, D	P3, D2	o
	19	P102L	MM	1	M	58	53	+	+	T	D	P3, D2	++
	20	P102L	MM	ND	M	64	24	—	+	—	T, D	S1, P1	o
Figure 1I-L	21	P102L	MM	ND	M	59	49	+++	+++	T, D	T, D	P3, D2	++
	22	P102L	MV	2	F	64	33	+++	++	D	D	S2, P3, D2	++
	23	P102L	MV	ND	M	61	50	+++	+++	T, D	T, D	S2, P3, D1	+++
	24	P102L	MM	2	F	65	64	+	++	T, D	T, D	S1, P3, D2	++
Figure 3E, F	25	P102L	MM	Negative	F	62	48	++	+	D	D	S2, P3, D1, PV1	+
	26	P102L	MM	Negative;8 kDa	M	52	155	+	+	D	D	P2	o
	27	P102L	MM	ND	F	66	81	+	+++	D	T, D	S1, P3	++
	28	6OPRI	MV	Negative	F	49	8	+++	+	T	D	S1	o
Figure 1E-H	29	6OPRI	MV	Negative	F	46	0	+++	+++	T	T, D	S2, P3, D3	o
	30	6OPRI	MM	ND	M	44	118	+++	+++	T, D	D	S3, GR1, P1, D1	+
Figure 3C, D, Figure 5A, B	31	6OPRI	MM	ND	F	45	72	+++	++	T	T, D	S1, PN1	+
	32	5OPRI	MM	ND	M	57	323	+	+	T, D	T, D	S1	+
	33	4OPRI	MM	ND	F	56	7	+++	+	T	T, D	S3, GR1	+
Figure 1A-D, Figure 3A, B	34	4OPRI	MM	ND	M	73	14	++	++	T, D	T, D	S1	+++
	35	4OPRI	MM	2	F	62	5	++	++	T	T, D	S1, GR1, PN1	++

*Proteinase K-digested PrP fragment size according to the London classification [14, 15].
ND, not done.
All case numbers (first column) are cross-referenced to Figures 1, 2, 3, 4, 5 and 6. The columns on the right describe the pattern of PrP deposition in the white matter. The semi quantitative density scores of white matter PrP are represented in column 8. mo: months, M: male/methionine, F: female, V: valine, WM: white matter, FB = Forebrain, Cer = Cerebellum, T = Threads, D = Dots. The two columns on the right describe the pattern and intensity of cortical PrP deposition as described in [11] and the degree of spongiform changes. S = synaptic, PN = perineuronal net, GR = Granular; P = well demarcated plaques, D = diffuse plaques; and PV = perivascular deposits. The number indicates the semi-quantitative intensity or density of the respective feature.

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ISSN: 2051-5960

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