Grant: Project Grant

Many diseases of the brain are caused by proteins that change their three-dimensional shape, and as a consequence form 'clumps' or aggregates in brain cells, eventually destroying the brain. Prion diseases such as Creutzfeldt-Jakob disease are the prototype group for such disorders. They are always fatal and cannot be treated to date. We found that cholesterol, an important component of cellular membranes, is elevated in prion-infected neurons, which favors the aggregation of the disease-causing protein. We propose to elucidate the consequences of elevated cholesterol levels and use a re-purposed pharmaceutical as a new approach for treatment of prion diseases.