Grant: Project Grant

Prions are the cause of deadly and uncurable brain diseases of humans and animals. Examples are Creutzfeldt-Jakob disease in humans or bovine spongiform encephalopathy (aka mad cow disease) in cattle. Prions are very special infectious agents because they consist only of protein, which builds up in clumps or aggregates with neurons and thereby, slowly destroy the brain. When zooming into prion-infected brain cells with a microscope, we can observe those protein aggregates only at specific sites - the cell surface and in internal, specialized membrane sacks or vesicles that distribute cargo to their specific destination within the cell. Our project will investigate how the presence of such protein aggregates affect the function of these vesicles, why some cell types in the brain such as neurons are affected differently than others, and how this dysfunction is linked to neurodegeneration. Eventually, our goal is to understand fundamental mechanisms of pathogenesis to define new treatment targets for those uncurable and deadly diseases.