California scientists have created a synthetic prion, a rogue protein that was used to infect mice with a brain destroying infection.
Prions are the cause of several fatal diseases, including BSE (bovine spongiform encephalitis) and CJG (Creutsfeldt-Jakob disease). It’s a nasty, nasty disease, which was chronicled in Richard Rhodes’ book Deadly Feasts. It’s not exactly comforting synthetic analogs of existing infectious prions have been synthesized in the laboratory. CJD is a disease with 100% fatality, and prions are immune to normal sterilization procedures with are effective against other infectious agents. Not good, not good.
It wasn’t known before this research was done whether prions, by themselves, could cause a disease or whether some other agent was required as well. This project settled the question. The more we know about these nasty molecules, the better.