The Mechanisms of Prion Disease

Gill, Andrew and Lane, Fiona M. and Alibhai, James and Manson, Jean C and McCutcheon, Sandra (2011) The Mechanisms of Prion Disease. CAB Reviews: Perspectives in Agriculture, Veterinary Science, Nutrition and Natural Resources, 6 . 055. ISSN 1749-8848

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Abstract

Prion diseases are enigmatic, neurodegenerative disorders affecting several mammalian species.
The family of diseases include scrapie of sheep, bovine spongiform encephalopathy (BSE),
Creutzfeldt-Jakob disease (CJD) of humans and chronic wasting disease (CWD) in deer and elk.
Prion diseases are believed to be caused by the misfolding of an endogenous host protein and the
abnormal, protease-resistant isoform of this protein is suggested to represent the infectious entity.
Through many transmission experiments, we know much about the course of prion diseases and
the ensuing pathology in the central nervous system. However, few of the causative mechanisms
have been deciphered at a level that allows prediction of the disease characteristics of novel prion
strains or that allows rational design of chemo-therapeutics. This review article outlines the state
of our knowledge of prion disease mechanisms from the concept of protein misfolding, through
the factors that may encode different strains of disease, mechanisms of neuronal loss and the ways
that disease are transmitted.

Keywords:transmissible spongiform encephalopathies, protein misfolding, disease transmission, prion strains, neurodegeneration, Neurotoxicity
Subjects:D Veterinary Sciences, Agriculture and related subjects > D320 Animal Health
B Subjects allied to Medicine > B140 Neuroscience
B Subjects allied to Medicine > B131 Cellular Pathology
Divisions:College of Science
ID Code:29474
Deposited On:25 Oct 2018 11:50

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