Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt-- Jakob disease: implications for human health.
There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.
Commissariat à l'Energie Atomique, Service de Neurovirologie, Direction des Sciences du Vivant/Département de Recherche Medicale, Centre de Recherches du Service de Santé des Armées 60-68, Fontenay-aux-Roses, France. lasmezas@cea.fr
C I
CI
Lasmézas
J G
JG
Fournier
V
V
Nouvel
H
H
Boe
D
D
Marcé
F
F
Lamoury
N
N
Kopp
J J
JJ
Hauw
J
J
Ironside
M
M
Bruce
D
D
Dormont
J P
JP
Deslys
D000220:Adaptation, Biological; D000818:Animals; D002417:Cattle; D007562:Creutzfeldt-Jakob Syndrome / Q000503:physiopathology / Q000635:transmission*; D004195:Disease Models, Animal; D016643:Encephalopathy, Bovine Spongiform / Q000503:physiopathology / Q000635:transmission*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D051379:Mice; D008810:Mice, Inbred C57BL; D010641:Phenotype; D018419:Primate Diseases / Q000635:transmission; D011323:Primates; D011328:Prions / Q000032:analysis*; D012608:Scrapie / Q000503:physiopathology
D011328:Prions