NDLI: Effects of a naturally occurring amino acid substitution in bovine PrP: a model for inherited prion disease in a natural host species

Content Provider	Springer Nature : BioMed Central
Author	Vrentas, Catherine E. Greenlee, Justin J. Foster, Gregory H. West, James Jahnke, Marianna M. Schmidt, Mark T. Nicholson, Eric M.
Abstract	Objective The most common hereditary prion disease is human Creutzfeldt-Jakob disease (CJD), associated with a mutation in the prion gene resulting in a glutamic acid to lysine substitution at position 200 (E200K) in the prion protein. Models of E200K CJD in transgenic mice have proven interesting but have limitations including inconsistencies in disease presentation, requirement for mixed species chimeric protein constructs, and the relatively short life span and time to disease onset in rodents. These factors limit research on the mechanism by which the mutation drives disease development. Therefore, our objective was to provide the first assessment of cattle carrying the homologous mutation, E211K, as a system for investigating longer-term disease mechanisms. The E211K substitution was associated with a case of bovine spongiform encephalopathy from 2006. Results We assessed the molecular properties of bovine E211K prion protein, characterized the molecular genetics of a population of cattle E211K carriers (offspring of the original EK211 cow) in relation to findings in humans, and generated preliminary evidence that the impacts of copper-induced oxidative stress may be different in cattle as compared to observations in transgenic mouse models. The cattle E211K system provides the opportunity for future analysis of physiological changes over time.
Related Links	https://bmcresnotes.biomedcentral.com/counter/pdf/10.1186/s13104-017-3085-8.pdf
Ending Page	6
Page Count	6
Starting Page	1
File Format	HTM / HTML
ISSN	17560500
DOI	10.1186/s13104-017-3085-8
Journal	BMC Research Notes
Issue Number	1
Volume Number	10
Language	English
Publisher	BioMed Central
Publisher Date	2017-12-20
Access Restriction	Open
Subject Keyword	Biomedicine Medicine Public Health Life Sciences Bos taurus PRNP Prion CJD Creutzfeldt-Jakob disease E200K E211K Cattle BSE PrP Medicine/Public Health
Content Type	Text
Resource Type	Article
Subject	Biochemistry, Genetics and Molecular Biology Medicine
Journal Impact Factor	1.6/2023

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