Prion diseases articles from across Nature Portfolio

This Primer by Zerr et al. discusses the epidemiology, pathophysiology, diagnosis and potential future treatments for Creutzfeldt–Jakob disease and other human prion diseases.

In this work, the authors investigated the cellular mode of prion propagation. The report that proteopathic seeds of abnormal PrP are N-terminally truncated and detected within minutes after infection. These seeds reach the plasma membrane by regulated secretory pathways where phenotypically distinct fibril-like PrP aggregates are formed with a lag of 24 h after infection.

A deep learning model rapidly identifies locality and counts of cored plaques and cerebral amyloid angiopathy in whole slide images comparably to human experts and without a GPU on a standard workstation.

A genome-wide association study has identified the first risk factors for sporadic prion disease other than mutations of PRNP, which encodes prion protein. These hits could open up new fronts in prion biology, risk prediction, and drug development.

A new study shows that interactions of the cellular prion protein with amyloid-β, tau and α-synuclein oligomers are important in mediating the toxicity of these proteins in Alzheimer disease and Parkinson disease. The findings suggest a shared pathway that could be a therapeutic target common to multiple neurodegenerative diseases.