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Generating a Prion with Bacterially Expressed Recombinant Prion Protein
Open accessAbstract
Recombinant Infectious Prions: Prion diseases are a group of fatal neurodegenerative disorders that include Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy in cows. The prion hypothesis states that the infectious agent of these diseases is an aberrant conformational isoform of the normal prion protein (PrP C ), a glycosylphosphatidylinositol-anchored cell surface protein enriched in the central nervous system. The final proof for the prion hypothesis is to convert bacterially expressed recombinant PrP into an infectious prion, but this has been difficult to achieve. F. Wang et al. (p. 1132 , published online 28 January; see the Perspective by Supattapone ) put recombinant PrP purified from bacteria into mice and obtained all the characteristics of the infectious agent in prion disease. The recombinant form is not only resistant to proteinase-K, but also shows infectivity in cultured cells and causes rapid disease progression in wild-type mice, yielding both the behavioral and the neuropathological symptoms.
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Cites
Prions and their partners in crime
Caughey, B., & Baron, G. S. (2006) · Nature · doi:10.1038/nature05294 · Cited by 295
Cited by
Self-propagation of pathogenic protein aggregates in neurodegenerative diseases
Jucker, M., & Walker, L. C. (2013) · Nature · doi:10.1038/nature12481 · Cited by 1,663
Degradation of misfolded proteins in neurodegenerative diseases: Therapeutic targets and strategies
Ciechanover, A., & Kwon, Y. T. (2015) · Experimental & Molecular Medicine · doi:10.1038/emm.2014.117 · Cited by 898
Cell-to-cell transmission of pathogenic proteins in neurodegenerative diseases
Guo, J. L., & Lee, V. M. Y. (2014) · Nature Medicine · doi:10.1038/nm.3457 · Cited by 641
Pathogenic protein seeding in Alzheimer disease and other neurodegenerative disorders
Jucker, M., & Walker, L. C. (2011) · Annals of Neurology · doi:10.1002/ana.22615 · Cited by 622
“It is possible that the aggregation of synthetic Ab under suitable (but as yet undefined) conditions will yield a more effective in vivo seed, as was recently demonstrated for PrP.(58) The differential seeding ability of synthetic versus natural Ab aggregates (which may contain additional factors) suggests the possibi…”
BackgroundBiology and Genetics of Prions Causing Neurodegeneration
Prusiner, S. B. (2013) · Annual Review of Genetics · doi:10.1146/annurev-genet-110711-155524 · Cited by 455
Metadata
- Authors
- Wang, F., Wang, X., Yuan, C.-G., & Ma, J.
- Venue
- Science
- Published
- 2010-02-26
- Volume
- 327
- Issue
- 5969
- Pages
- 1132-1135
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2015329538
- Cited by
- 661
- References
- 1
- FWCI
- 33.55
- Type
- journal article
- Language
- en
- Open access
- green
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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