8DJA

Cryo-EM structure of mouse PrP23-144 amyloid fibrils (polymorph 1)

Summary for 8DJA

Entry DOI	10.2210/pdb8dja/pdb
EMDB information	27458
Descriptor	Major prion protein (1 entity in total)
Functional Keywords	moprp23-144, amyloid, prion diseases, protein fibril
Biological source	Mus musculus (house mouse)
Total number of polymer chains	20
Total formula weight	247971.58
Authors	Li, Q.,Surewicz, W.K. (deposition date: 2022-06-30, release date: 2022-09-07, Last modification date: 2024-06-12)
Primary citation	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 Cited by 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 PDB entries with the same primary citation
Experimental method	ELECTRON MICROSCOPY (3.92 Å)

Structure validation