Prion strains viewed through the lens of cryo-EM.
Cryo-EM
Prion
Prion disease
Prion strains
Prion structure
Journal
Cell and tissue research
ISSN: 1432-0878
Titre abrégé: Cell Tissue Res
Pays: Germany
ID NLM: 0417625
Informations de publication
Date de publication:
Apr 2023
Apr 2023
Historique:
received:
28
05
2022
accepted:
18
08
2022
medline:
20
4
2023
pubmed:
27
8
2022
entrez:
26
8
2022
Statut:
ppublish
Résumé
Mammalian prions are lethal transmissible pathogens that cause fatal neurodegenerative diseases in humans and animals. They consist of fibrils of misfolded, host-encoded prion protein (PrP) which propagate through templated protein polymerisation. Prion strains produce distinct clinicopathological phenotypes in the same host and appear to be encoded by distinct misfolded PrP conformations and assembly states. Despite fundamental advances in our understanding of prion biology, key knowledge gaps remain. These include precise delineation of prion replication mechanisms, detailed explanation of the molecular basis of prion strains and inter-species transmission barriers, and the structural definition of neurotoxic PrP species. Central to addressing these questions is the determination of prion structure. While high-resolution definition of ex vivo prion fibrils once seemed unlikely, recent advances in cryo-electron microscopy (cryo-EM) and computational methods for 3D reconstruction of amyloids have now made this possible. Recently, near-atomic resolution structures of highly infectious, ex vivo prion fibrils from hamster 263K and mouse RML prion strains were reported. The fibrils have a comparable parallel in-register intermolecular β-sheet (PIRIBS) architecture that now provides a structural foundation for understanding prion strain diversity in mammals. Here, we review these new findings and discuss directions for future research.
Identifiants
pubmed: 36028585
doi: 10.1007/s00441-022-03676-z
pii: 10.1007/s00441-022-03676-z
pmc: PMC10113314
doi:
Substances chimiques
Prions
0
Prion Proteins
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
167-178Subventions
Organisme : Medical Research Council
ID : MC_U123192748
Pays : United Kingdom
Organisme : Medical Research Council
ID : MC_UU_00024/5
Pays : United Kingdom
Organisme : Medical Research Council
ID : MC_UU_00024/5
Pays : United Kingdom
Informations de copyright
© 2022. The Author(s).
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