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Variant Creutzfeldt-Jakob disease
H J T Ward1, M W Head, R G Will
1National Creutzfeldt-Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK.
Variant Creutzfeldt-Jakob disease (vCJD) is a new human prion disease transmitted from animals. Research is ongoing to understand transmission, exposure, and potential treatments like quinacrine or chloropromazine.
Area of Science:
- Neuroscience
- Infectious Diseases
- Public Health
Background:
- Variant Creutzfeldt-Jakob disease (vCJD) is a novel human prion disease.
- It is the first documented instance of animal prion diseases transmitting to humans.
- Significant uncertainties surround vCJD, including transmission mechanisms and human exposure levels.
Purpose of the Study:
- To address uncertainties regarding vCJD transmission and its public health implications.
- To explore potential therapeutic avenues for human prion diseases.
- To investigate the mechanism of prion replication for treatment development.
Main Methods:
- Review of existing scientific literature on vCJD and prion diseases.
- Analysis of epidemiological data related to Bovine Spongiform Encephalopathy (BSE) and vCJD.
- Exploration of recent pharmacological studies on potential treatments.
Main Results:
- vCJD represents a zoonotic transmission of prion disease.
- Key uncertainties include interspecies transmission, human exposure to BSE, infectious dose, and future disease burden.
- Recent research suggests quinacrine and chloropromazine as potential treatments for human prion diseases.
Conclusions:
- Further scientific research is crucial to answer outstanding questions about vCJD.
- Understanding prion replication mechanisms may pave the way for effective treatments.
- Quinacrine and chloropromazine show promise as candidate therapies for human prion diseases.
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