Prion strains viewed through the lens of cryo-EM

Manka Szymon W.; Wenborn Adam; Collinge JohnWadsworth Jonathan D. F.

首页> 外文期刊>Cell and Tissue Research >Prion strains viewed through the lens of cryo-EM

【24h】

Prion strains viewed through the lens of cryo-EM

机译：Prion strains viewed through the lens of cryo-EM

获取原文

获取原文并翻译 | 示例

掌桥外文数据库（机构版） >>

开具论文收录证明 >>

文献代查 >>

页面导航

摘要
著录项
相关主题

摘要

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 beta-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.

著录项

来源
《Cell and Tissue Research》 |2023年第1期|167-178|共12页
作者
Manka Szymon W.; Wenborn Adam; Collinge JohnWadsworth Jonathan D. F.;
展开▼
作者单位

University of London,UCL;

展开▼
收录信息
原文格式 PDF
正文语种英语
中图分类细胞生物学;
关键词
Cryo-EM; Prion; Prion disease; Prion strains; Prion structure; STRAUSSLER-SCHEINKER-DISEASE; CREUTZFELDT-JAKOB-DISEASE; N-LINKED GLYCANS; AMYLOID FIBRILS; VARIANT CJD; PHENOTYPIC HETEROGENEITY; PROTEIN ISOFORMS; WILD-TYPE; PRP; PARALLEL;

Prion strains viewed through the lens of cryo-EM

摘要

著录项

相关主题

期刊订阅