Browse by author
Lookup NU author(s): Professor Tiago OuteiroORCiD
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).
© 2024 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.The history of human prion diseases began with the original description, by Hans Gerhard Creutzfeldt and by Alfons Maria Jakob, of patients with a severe brain disease that included speech abnormalities, confusion, and myoclonus, in a disease that was then named Creutzfeldt Jakob disease (CJD). Later, in Papua New Guinea, a disease characterized by trembling was identified, and given the name “Kuru”. Neuropathological examination of the brains from CJD and Kuru patients, and of brains of sheep with scrapie disease revealed significant similarities and suggested a possible common mode of infection that, at the time, was thought to derive from an unknown virus that caused slow infections. John Stanley Griffith hypothesized that the agent causing these diseases was “probably a protein without nucleic acid” and, in 1982, Stanley Prusiner reported the identification of a proteinaceous infectious particle (coining the term prion) that was resistant to inactivation methods that were at the time standard for nucleic acids, and identified PrP as the major protein component of the infectious agent in scrapie and in Creutzfeldt-Jakob disease, classifying this also as a prion disease. Interestingly, the prion concept had been previously expanded to yeast proteins capable of replicating their conformation, seeding their own aggregation and transmitting phenotypic information. The prion concept has been more recently expanded to refer to misfolded proteins that are capable of converting a normal form of a protein into an abnormal form. The quest to understand and treat prion diseases has united a specific research community around the topic, and regular meetings (Prion Meetings) have taken place over the years to enable discussions, train junior researchers, and inspire research in the field.
Author(s): Outeiro TF, Vieira TCRG
Publication type: Letter
Publication status: Published
Journal: Prion
Year: 2024
Volume: 18
Issue: 1
Pages: 68-71
Online publication date: 23/04/2024
Acceptance date: 07/02/2024
ISSN (print): 1933-6896
ISSN (electronic): 1933-690X
Publisher: Taylor and Francis Ltd.
URL: https://doi.org/10.1080/19336896.2024.2343535
DOI: 10.1080/19336896.2024.2343535
PubMed id: 38651736
Data Access Statement: Data sharing is not applicable to this article as no new data were created or analysed in this study
