Open AccessRecent advances on the molecular pathogenesis of prion diseases
Author(s) -
Glatzel Markus,
Sigurdson Christina J.
Publication year - 2019
Publication title -
brain pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.986
H-Index - 132
eISSN - 1750-3639
pISSN - 1015-6305
DOI - 10.1111/bpa.12693
Subject(s) - chronic wasting disease , kuru , bovine spongiform encephalopathy , fatal familial insomnia , disease , transmissible spongiform encephalopathy , scrapie , virology , creutzfeldt jakob syndrome , slow virus , prion protein , infectious agent , infectious disease (medical specialty) , neurodegeneration , biology , pathogenesis , medicine , immunology , pathology , virus , viral disease
Prion diseases continue to fascinate scientists from different disciplines since the discovery that a misfolded prion protein can act as an infectious agent. Infectious prions have caused epidemics, including kuru in humans, cervid chronic wasting disease, bovine spongiform encephalopathy (BSE) or "mad cow disease", and most recently, camel prion disease, which was identified in 2018 (1). In Kuru, spread of the disease occurred from the ingestion of prion-infected, dead relatives as part of mourning practices, whereas in chronic wasting disease, prions may be transmitted not only through ingesting prion-contaminated food, but also by exposure to a prion-contaminated environment(8, 9). In all instances, prions hold structural properties that cause different disease presentations, which can be passed on to another individual (16). This article is protected by copyright. All rights reserved.