The Barron Group’s research is based around prion diseases, specifically ones such as bovine spongiform encephalopathy (BSE, or Mad Cow Disease). A prion disease is a specific type of disorder in which a protein in the brain (PrP) misfolds and aggregates, and gets deposited in the brain, destroying the brain cells. Or at least that is the hypothesis. There is no a bacterium or virus which is known to cause the misfolding of the protein, which begs the question, exactly what does? It is this question we hope to answer. 

Prion diseases are neurodegenerative diseases. These diseases are invariably fatal and there is no current cure or preventative treatment. Once clinical signs of disease begin to show, death in inevitable. Prion diseases such as BSE have been controlled, not eradicated. Incidence of BSE has declined in the UK, but a handful of cases are still being identified. New atypical forms of BSE and scrapie have been identified over the last decade. Chronic wasting disease (CWD) is a prion disease of deer. This has previously been restricted to North America, but cases have recently been identified in Europe. While these diseases remain in the environment, risks to human and animal health remain.

In our research to identify the cause of disease, we use a technique which involves injecting prion infected brain tissue into the brain of normal or genetically engineered mice. This causes the mouse to become infected and samples can be taken and screened for signs of disease. Brain tissue is taken and examined for specific forms of the prion protein (PrP) which are known to be associated with disease. Normally in prion infected mice a specific misfolded form of PrP can be identified by separation on gels or in slices of brain. This protein is a marker of disease and thought to be the infectious agent. However, samples taken from unusual models of prion disease in the Barron Lab showed little or no evidence of this abnormal form of the prion protein, even though the mice had clinical signs of prion disease. Because diagnostic tests rely on being able to detect abnormal PrP, can these tests always be relied upon? We aim to use these unusual models to identify what is causing disease in these animals, and identify better biomarkers of infection.