Bona fide atypical scrapie faithfully reproduced for the first time in a rodent model.
Atypical
Isoleucine
Prion disease
Scrapie
Spontaneous
Journal
Acta neuropathologica communications
ISSN: 2051-5960
Titre abrégé: Acta Neuropathol Commun
Pays: England
ID NLM: 101610673
Informations de publication
Date de publication:
13 12 2022
13 12 2022
Historique:
received:
16
09
2022
accepted:
10
11
2022
entrez:
13
12
2022
pubmed:
14
12
2022
medline:
16
12
2022
Statut:
epublish
Résumé
Atypical Scrapie, which is not linked to epidemics, is assumed to be an idiopathic spontaneous prion disease in small ruminants. Therefore, its occurrence is unlikely to be controlled through selective breeding or other strategies as it is done for classical scrapie outbreaks. Its spontaneous nature and its sporadic incidence worldwide is reminiscent of the incidence of idiopathic spontaneous prion diseases in humans, which account for more than 85% of the cases in humans. Hence, developing animal models that consistently reproduce this phenomenon of spontaneous PrP misfolding, is of importance to study the pathobiology of idiopathic spontaneous prion disorders. Transgenic mice overexpressing sheep PrP
Identifiants
pubmed: 36514160
doi: 10.1186/s40478-022-01477-7
pii: 10.1186/s40478-022-01477-7
pmc: PMC9749341
doi:
Substances chimiques
Prions
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
179Subventions
Organisme : NIAID NIH HHS
ID : P01 AI077774
Pays : United States
Informations de copyright
© 2022. The Author(s).
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