Project Details

Description

Development and comparison of prion amplification assays (PMCA and RT-QuIC) to detect prion infection in blood samples from a large animal model of vCJD i.e. sheep infected experimentally with BSE.

Layman's description

Aim
This project aimed to develop laboratory tests for variant Creutzfeldt-Jakob disease (vCJD) that could accurately detect the disease at an early stage of infection, long before an affected individual becomes ill. Such tests could potentially be of great benefit in protecting the public from further spread of the disease.

Background
Ideally, a laboratory test for vCJD should be able to work on patient samples that are non-invasive and easy to collect, such as blood or urine. Such a test could then be used for early diagnosis, or to screen donated blood for vCJD. It could also help us to identify ‘carriers’ of the disease, who might be at risk of infecting others.
In this research project, we used sheep infected experimentally with BSE to study the risk of transmitting prion diseases by transfusion of blood components commonly used in humans. Sheep were used for these studies because there were very few suitable human blood samples available to optimise our new diagnostic tests. Sheep also show many similarities to human vCJD patients when they are infected with BSE, and are a similar size to humans. Blood samples from infected sheep were collected and frozen at regular intervals, from the time they were infected until they developed signs of brain disease 2-4 years later. Blood samples from the sheep model were then used to determine which diagnostic tests were best at detecting subclinical infection.

Outcome
Three potential diagnostic platforms were tested and their performance compared. All three were capable of detecting early (preclinical) infection, with varying levels of sensitivity. Furthermore, we have data to suggest that the blood tests developed in this project could be further adapted for use in humans. We hope that the outcomes of this study will help us to better understand the risks of human-to-human transmission and allow us to devise policies to minimise those risks.

Key findings

A detailed comparison of the sensitivity and specificity of three putative blood tests for prion disease. Additional data that suggests these tests could be further adapted for use in humans.
StatusFinished
Effective start/end date1/03/1831/08/21

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