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Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
Published on: January 8, 2015
Transgenic models of prion disease
M R Scott1, S Supattapone, H O Nguyen
1Institute for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco 94143-0518, USA.
Archives of Virology. Supplementum
|February 24, 2001
Summary
Bovine spongiform encephalopathy (BSE) prions transmit to humans, causing variant Creutzfeldt-Jakob disease (vCJD). Transgenic mice expressing bovine PrP show no species barrier, supporting human transmission. Miniprions offer insights into prion replication.
Area of Science:
- Neuroscience
- Molecular Biology
- Prion Diseases
Background:
- Growing concern exists regarding bovine spongiform encephalopathy (BSE) transmission to humans, potentially causing variant Creutzfeldt-Jakob disease (vCJD).
- Understanding prion propagation and species barriers is crucial for public health and disease modeling.
Purpose of the Study:
- To investigate the transmission of BSE prions from cattle to humans using transgenic mouse models.
- To explore the role of prion protein (PrP) sequences in prion formation and replication.
- To develop novel animal models for studying prion diseases.
Main Methods:
- Generation of transgenic (Tg) mice expressing full-length bovine (Bo) PrP and a redacted form (PrP106).
- Inoculation of Tg mice with BSE, vCJD, and natural sheep scrapie prions.
- Analysis of incubation times, neuropathology, and PrP(Sc) isoforms.
Main Results:
- Tg(BoPrP) mice efficiently propagated BSE prions with no species barrier from cattle.
- Tg(BoPrP) mice showed indistinguishable disease characteristics when inoculated with BSE and vCJD.
- A redacted prion protein (PrP106) supported prion propagation, and its 'miniprions' replicated efficiently in Tg(PrP106) mice, especially with coexpressed full-length mouse PrP.
Conclusions:
- Findings provide compelling evidence for BSE transmission to humans, causing vCJD.
- Miniprions exhibit unique replication features, offering new insights into prion disease mechanisms.
- Coexpression of full-length PrP suggests a novel approach for developing enhanced animal models for prion diseases.
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