Prions: both a blessing and a curse

The prion protein is important for the health of nerve cells in the brain. When it changes its shape, it can cause incurable diseases of the nervous system.

The prion may be one of the most exciting medical discoveries of the past 35 years. These tiny proteins produced by our own bodies play a role in maintaining healthy nerve cells in the brain that has as yet not been extensively researched. But prions have the capacity to become malignant overnight simply by changing their shape. This can be caused by a transmitted defect in the human genome, or when a healthy prion comes into contact with a modified one, for example through the ingestion of BSE-contaminated meat.

Such altered prions kill nerve cells, which leads to diseases such as Creutzfeldt-Jakob disease, the human form of “mad cow's disease”. Prions also play a role in the development of Alzheimer's and Parkinson's. Adriano Aguzzi, neuropathologist at the University Hospital of Zurich, is looking for the genes responsible for the production of prions. He has already identified one of them. "It is exciting that we can control the activity of this gene with drugs that are available to us," says Aguzzi. "Our hope is that we can find a therapy for Creutzfeldt-Jakob disease based on this, and perhaps even for Alzheimer's and Parkinson's."

Contact: Adriano Aguzzi, adriano.aguzzi(at), +41 44 255 21 07


Department program of SNF