Discovery of prion

The study of prions started with investigation into the nature of the transmissible agent causing fatal neurological illnesses affecting humans and animals.

The oldest prion disease known is scrapie, which occurs naturally in goat and sheep, Although the disease was recognized as far back 1752, the first scientific description dates from 1818.

Prion is a term coined by Stanley Prusiner in 1982 to describe the infectious material fund in animals suffering from the disease scrapie, but is now used more broadly to mean infectious in general. The word prion derives form ‘proteinaceous infectious particle’.

Prusiner was a professor of neurology and biochemistry at the University of California School of Medicine, San Francisco. He began the studies of prions in the early 1970s. He won a Nobel Prize in 1997 for his discovery.

According to Prusiner, prion is not a virus or bacterium, but a protein devoid of nucleic acids and RNA.

The discovery provided a mechanism for understanding mad cow disease or bovine spongiform encephalopathy, a serious problem in post-industrial societies. It also provides key insight into dementia related diseases like Alzheimer’s.

A particularly strong piece of evidence in favor of the prion hypothesis came from the group of Charles Weismann in 1993 who showed that mice lacking the protease-resistant prion protein gene were resistant to scrapie infection, neither developing signs of scrapie nor allowing propagation of the disease.
Discovery of prion