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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 ++
  1. *Proteinase K-digested PrP fragment size according to the London classification [14, 15].
  2. ND, not done.
  3. 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.