A worldwide group of scientists has created an infectious prion disease in a mouse model, in a step that may help unravel the mystery of this progressive disease that affects the nervous system in humans and animals.
The research team, including Christina J. Sigurdson, D.V.M., Ph.D., assistant professor of pathology at the University of California, San Diego School of Medicine, also discovered that changing the structure of the prion protein by altering just two nucleic acids leads to a fatal neurological disorder in mice. Their findings will be published on line in Proceedings of the National Academy of Sciences ( PNAS ) the week of December 1.
The study, led by Professor Dr. Adriano Aguzzi of the Institute of Neuropathology at the University of Zurich in Switzerland, was designed to investigate the specific changes in the prion protein that may contribute to chronic wasting disease (CWD). CWD is a highly infectious prion disease found in free-ranging deer and elk that is similar to bovine spongiform encephalopathy (BSE, or "mad cow disease") in cattle and Creutzfeldt-Jakob disease in humans. Prion diseases are thought to be a result of a misfolded form of the prion protein that induces formation of amyloid plaques in the brain – changes that are also seen in patients with Alzheimer's disease.