Gene-edited cell models to study chronic wasting disease
Gene-edited cell models to study chronic wasting disease
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Full item details
- creativework.keywords - en
- Prion disease
- Bovine spongiform encephalopathies
- creativework.keywords - fr
- Maladies à prions
- Encéphalopathies spongiformes bovines
- dc.contributor.author
- Thapa, Simrika
- Marrero Winkens, Cristobal
- Tahir, Waqas
- Arifin, Maria I.
- Gilch, Sabine
- Schatzl, Hermann M.
- dc.date.accepted
- 2022-03-11
- dc.date.accessioned
- 2025-04-09T17:13:51Z
- dc.date.available
- 2025-04-09T17:13:51Z
- dc.date.issued
- 2022-03-15
- dc.date.submitted
- 2022-02-08
- dc.description.abstract - en
- Prion diseases are fatal infectious neurodegenerative disorders affecting both humans and animals. They are caused by the misfolded isoform of the cellular prion protein (PrPC), PrPSc, and currently no options exist to prevent or cure prion diseases. Chronic wasting disease (CWD) in deer, elk and other cervids is considered the most contagious prion disease, with extensive shedding of infectivity into the environment. Cell culture models provide a versatile platform for convenient quantification of prions, for studying the molecular and cellular biology of prions, and for performing high-throughput screening of potential therapeutic compounds. Unfortunately, only a very limited number of cell lines are available that facilitate robust and persistent propagation of CWD prions. Gene-editing using programmable nucleases (e.g., CRISPR-Cas9 (CC9)) has proven to be a valuable tool for high precision site-specific gene modification, including gene deletion, insertion, and replacement. CC9-based gene editing was used recently for replacing the PrP gene in mouse and cell culture models, as efficient prion propagation usually requires matching sequence homology between infecting prions and prion protein in the recipient host. As expected, such gene-editing proved to be useful for developing CWD models. Several transgenic mouse models were available that propagate CWD prions effectively, however, mostly fail to reproduce CWD pathogenesis as found in the cervid host, including CWD prion shedding. This is different for the few currently available knock-in mouse models that seem to do so. In this review, we discuss the available in vitro and in vivo models of CWD, and the impact of gene-editing strategies.
- dc.identifier.citation
- Thapa, S., Marrero Winkens, C., Tahir, W., Arifin, M. I., Gilch, S., & Schatzl, H. M. (2022). Gene-edited cell models to study chronic wasting disease. Viruses, 14(3), 609. https://doi.org/10.3390/v14030609
- dc.identifier.doi
- https://doi.org/10.3390/v14030609
- dc.identifier.issn
- 1999-4915
- dc.identifier.uri
- https://open-science.canada.ca/handle/123456789/3597
- dc.language.iso
- en
- dc.publisher - en
- MDPI
- dc.rights - en
- Creative Commons Attribution 4.0 International (CC BY 4.0)
- dc.rights - fr
- Creative Commons Attribution 4.0 International (CC BY 4.0)
- dc.rights.uri - en
- https://creativecommons.org/licenses/by/4.0/
- dc.rights.uri - fr
- https://creativecommons.org/licenses/by/4.0/deed.fr
- dc.subject - en
- Animal diseases
- Diseases
- dc.subject - fr
- Maladie animale
- Maladie
- dc.subject.en - en
- Animal diseases
- Diseases
- dc.subject.fr - fr
- Maladie animale
- Maladie
- dc.title - en
- Gene-edited cell models to study chronic wasting disease
- dc.type - en
- Article
- dc.type - fr
- Article
- local.acceptedmanuscript.articlenum
- 609
- local.article.journalissue
- 3
- local.article.journaltitle - en
- Viruses
- local.article.journalvolume
- 14
- local.peerreview - en
- Yes
- local.peerreview - fr
- Oui
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