7RL4

Cryo-EM structure of human PrP23-144 amyloid fibrils

7RL4 の概要

• エントリーDOI: 10.2210/pdb7rl4/pdb
• EMDBエントリー: 24514
• 分子名称: Major prion protein (1 entity in total)
• 機能のキーワード: prion, amyloid, protein fibril
• 由来する生物種: Homo sapiens (Human)
• タンパク質・核酸の鎖数: 20
• 化学式量合計: 250834.86

構造登録者

Li, Q.,Surewicz, W.K. (登録日: 2021-07-23, 公開日: 2022-07-27, 最終更新日: 2024-06-05)

主引用文献

Li, Q.,Jaroniec, C.P.,Surewicz, W.K.
Cryo-EM structure of disease-related prion fibrils provides insights into seeding barriers.
Nat.Struct.Mol.Biol., 29:962-965, 2022

PubMed Abstract: One of the least understood aspects of prion diseases is the structure of infectious prion protein aggregates. Here we report a high-resolution cryo-EM structure of amyloid fibrils formed by human prion protein with the Y145Stop mutation that is associated with a familial prion disease. This structural insight allows us not only to explain previous biochemical findings, but also provides direct support for the conformational adaptability model of prion transmissibility barriers.

PubMed: 36097290
DOI: 10.1038/s41594-022-00833-4

実験手法

ELECTRON MICROSCOPY (2.86 Å)