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Volume 23, Number 6—June 2017


Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients

Jean Y. Douet, Caroline Lacroux, Naima Aron, Mark W. Head, Séverine Lugan, Cécile Tillier, Alvina Huor, Hervé Cassard, Mark Arnold, Vincent Beringue, James W. Ironside, and Olivier AndréolettiComments to Author 
Author affiliations: Institut National de la Recherche Agronomique, Toulouse, France (J.Y. Douet, C. Lacroux, N. Aron, S. Lugan, C. Tillier, A. Huor, H. Cassard, O. Andréoletti); University of Edinburgh, Edinburgh, Scotland, UK (M.W. Head, J.W. Ironside); Animal and Plant Health Agency, Loughborough, UK (M. Arnold); Institut National de la Recherche Agronomique, Jouy-en-Josas, France (V. Beringue)

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Table 5

Bone marrow sample bioassay in bovine PrP–expressing mice (tgBov) for 4 patients with vCJD*

Sample Transmission in tgBov mice
Mean infectious titer, LD50/g (95% CI)
No. positive/no. inoculated mice Mean ± SD incubation, d
vCJD-1 5/5 458 ± 37 103.1 (102.6–103.5)
vCJD-2 6/6 373 ± 35 104.7 (104.3–05.2)
vCJD-3 4/6 504 ± 10 102.3 (101.8–102.7)
vCJD-4 6/6 447 ± 91 104.0 (103.4–04.5)
PBS control 0/6 >600 NA

*A 10% wt/vol bone marrow homogenate prepared from 4 symptomatic vCJD patients (Table 3) was inoculated intracerebrally into 6 tgBov mice (20 µL/mouse). One mouse (inoculated with homogenate from patient vCJD-1) died within the first few days after intercerebral inoculation. Mice were euthanized when they showed clinical signs of prion infection or after 600-d postinoculation. Mice were considered prion infected when abnormal PrP deposition was detected in brain. Infectious prion titers were estimated by using the method of Arnold et al. (17). The method uses the probability of survival (attack rate at each dilution) and the individual mouse incubation periods at each dilution to estimate infectious load. Infectious titers are given as estimated values. LD50, 50% lethal dose; NA, not applicable; PBS, phosphate-buffered saline; PrP; prion protein; vCJD, variant Creutzfeldt-Jakob disease.

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