Em Unit, Vla/Science Photo Library
I was at my laboratory bench one morning in 1980 when a colleague walked in and declared that he had identified the cause of scrapie, a mysterious and fatal infection that leaves tiny holes in the brains of sheep and goats. Stanley Prusiner had been studying the disease for some years and was stirring up controversy with his outlandish claim that the scrapie agent lacked genes or indeed any genetic material. It was, he said, an infectious protein – something never heard of before.
His issue that morning was what to call this unique protein. He had two candidates: “piaf” and “prion”. I have forgotten what piaf stood for, but I remember pointing out that the name was already taken by a popular French singer. Fine, he said, in any case he preferred prion, a contraction of protein and infection. I agreed. What I didn’t say was that in my native French tongue prions means “let us pray” – and that if he persisted with his idea of infectious proteins, he would need prayers.
Prusiner held strong in the face of adversity and, in 1997, won a Nobel prize for his discovery. By then, prions had been linked to Creutzfeldt-Jakob disease (CJD) in humans and to bovine spongiform encephalopathy or “mad cow disease”. There were also suggestions that they were involved in common neurodegenerative diseases including Alzheimer’s. What nobody predicted was the existence of “good” prions. We now know that prions emerged early in the evolution of life and play essential biological roles, from giving yeasts the ability to rapidly adapt to allowing you to form long-term memories.
The story of prions …
