A new treatment route for bovine spongiform encephalopathy (BSE) and its human form Creutzfeldt Jakob disease (CJD) could be a step closer thanks to scientists at the University of Leeds.
The team have found that a protein called Glypican-1 plays a key role in the development of BSE - otherwise known as Mad Cow Disease.
BSE is known to be caused by an infectious and abnormal form of the prion protein which is present on cells within the nervous system. But scientists have been unclear as to what causes the prions to become abnormal.
The new research from Leeds' Faculty of Biological Sciences provides part of the answer. The researchers have shown that the presence of Glypican-1 causes the numbers of abnormal prion proteins to rise. In experiments, when levels of Glypican-1 were reduced in infected cells, the levels of the abnormal prion reduced as well.
The discovery was a mixture of scientific detective work and luck, according to Professor of Biochemistry, Nigel Hooper.
"We were looking at how the normal prion protein functions in cells and spotted that it was interacting with something," he said. "Some lateral thinking and deduction led us to Glypican-1 and when we carried out the experiment, we found we were right."
The team believe that Glypican-1 acts as a scaffold bringing the two forms of the prion protein together and that this contact causes normal prions to mutate into the infectious form. They are seeking further funding to investigate their hypothesis.
The findings have implications for the treatment of both BSE and the human form of the disease, CJD, according to Professor Hooper.
"Now that we know the identity of one of the key molecules in the disease process, we may in the future be able to design drugs that target this."
Although the scientists mainly conducted experiments using cells infected with prions, it's also possible that Glypican-1 is involved in other diseases of the nervous system.
"While initial experiments haven't shown any link with other neurodegenerative diseases like Alzheimer's, we're not yet completely ruling that out," said Professor Hooper.
The research is published today (November 20) in PLoS Pathogens and was mainly funded through the Wellcome Trust with additional support from the Medical Research Council.
Professor Hooper is available for interview.
A copy of the paper is available on request.
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Notes for editors
1. The Faculty of Biological Sciences at the University of Leeds is one of the largest in the UK, with over 150 academic staff and over 400 postdoctoral fellows and postgraduate students. The Faculty is ranked 4th in the UK (Nature Journal, 457 (2009) doi :10.1038/457013a) based on results of the 2008 Research Assessment Exercise (RAE). The RAE feedback noted that "virtually all outputs were assessed as being recognized internationally, with many (60%) being internationally excellent or world-leading" in quality. The Faculty's research grant portfolio totals some £60M and funders include charities, research councils, the European Union and industry.
2. The 2008 Research Assessment Exercise showed the University of Leeds to be the UK's eighth biggest research powerhouse. The University is one of the largest higher education institutions in the UK and a member of the Russell Group of research-intensive universities. The University's vision is to secure a place among the world's top 50 by 2015.
3. For almost 100 years the Medical Research Council has improved the health of people in the UK and around the world by supporting the highest quality science. The MRC invests in world-class scientists. It has produced 29 Nobel Prize winners and sustains a flourishing environment for internationally recognised research. The MRC focuses on making an impact and provides the financial muscle and scientific expertise behind medical breakthroughs, including the first antibiotic penicillin, the structure of DNA and the lethal link between smoking and cancer. Today MRC funded scientists tackle research into the major health challenges of the 21st century.
4. The Wellcome Trust is the largest charity in the UK. It funds innovative biomedical research, in the UK and internationally, spending over £600 million each year to support the brightest scientists with the best ideas. The Wellcome Trust supports public debate about biomedical research and its impact on health and wellbeing.