The rogue prion proteins responsible for diseases like BSE and vCJD can destroy brain cells by blocking the protein recycling process that helps keep cells healthy.
An international group of scientists, led by Dr Sarah Tabrizi of University College London's Institute of Neurology, has now discovered which part of the structure of the protein it is that creates this effect and that even a tiny number of rogue prions are toxic enough to stop a cell's protein clearing machinery and cause disease.
Together with colleagues at the Medical Research Council Prion Unit, Dr Tabrizi's team monitored the action of rogue prion proteins on the brain cells of mice genetically designed for the task. The brain cells of these mice produce a protein that glows green when the cell's recycling machinery is failing allowing scientists to assess how the system has been affected. Their results are published in Molecular Cell .
A comparison of mouse nerve cells infected with and without prions, the green glowing patches develop as the cell's recycling machinery fails in prion infected cells.