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Table 2 CSF RT-QuIC protocols and diagnostic accuracy in prion disease

From: Towards an improved early diagnosis of neurodegenerative diseases: the emerging role of in vitro conversion assays for protein amyloids

PrP Substrate Reaction buffer Parameters Cohort size (N)a Sens. Spec. Ref.
Hu 23–231 0.06–0.1 g/L substrate, 15 μL seed, 500 mM NaCl 30 s circular shaking, 30 s incubation, 2 min break, 37 °C 18 def sCJD 83%   [5]
35 def non-CJD   100%
25 prob. sCJD 100%  
130 ctrl   100%
16 prob. sCJD 87%  
14 ctrl   100%
0.05 g/L substrate, 5 μL seed, 500 mM NaCl 24 gCJD 83% - [81]
20 GSS 90% -
12 FFI 83% -
Ha 23–231 0.1 g/L substrate, 15 μl seed, 300 mM NaCl 1 min double orbital shaking 600 rpm, 1 min rest, 42 °C cohort 1: 56 def sCJD 91%   [54]
52 ctrl   98%
cohort 2: 67 def sCJD 87%   
52 ctrl   100%  
15/13 def/prob. sCJD 79%   [59]
43 ctrl   100%
179/97/29 def/prob./pos sCJD 81%   [50]
46 gCJD 91%  
348 ctrl   99%
24/26 def/prob. sCJD 73% [13]
Ha 14–128 - Sheep 141–234 (chimera) 0.1 g/L substrate, 15 μL seed, 170 mM NaCl 1 min double orbital shaking, 600 rpm, 1 min rest, 42 °C 64 def sCJD 80%   [22]
39 gCJD 100%  
400 ctrl   99%
Ha 90–231 0.1 g/L substrate, 15–30 μL seed, 300 mM NaCl, 0,002% SDS 1 min double orbital shaking 700 rpm, 1 min rest, 55 °C 10/1 def/prob. sCJD 91% - [60]
43/5 def/prob. sCJD 96%  
39 ctrl   100%
36/68 def/prob. sCJD 94%   [36]
64 ctrl   100%
111 sCJD 92%   
15 gCJD 93%   [30]
67 ctrl   98.5%  
63 sCJD, 14 ctrlb 95% 100%  
10/12 def/prob. sCJD 95%   [13]
17 ctrl   100%
116/73 def/prob. sCJD 93%   [31]
33 gCJD 100%  
100 ctrl   100%
61/41 def/prob. sCJD 96%   [29]
80 ctrl   100%
439 def sCJD 93   [72]
31 gCJD 97%  
69 ctrl   98.5%
  1. Abbreviations: Hu Human, Ha Hamster, prob Probable, pos Possible, def Definite, ctrl Controls, sens. Sensitivity, spec. Specificity, sCJD Sporadic CJD, gCJD Genetic CJD, FFI Fatal familial insomnia, GSS Gerstmann-Sträussler-Scheinker disease
  2. aResults obtained in disease groups with an n of 9 or lower have been omitted
  3. bProspective cohort