A
prion () is an infectious agent thought to be the cause of the
transmissible spongiform encephalopathies (TSEs). It is composed entirely of protein material, called
PrP (short for prion protein), that can
fold in multiple, structurally distinct ways, at least one of which is transmissible to other prion proteins, leading to disease that is similar to viral infection. The word
prion, coined in 1982 by
Stanley B. Prusiner—derived from the words
protein and infect
ion, hence
prion—is short for "proteinaceous infectious particle", in reference to its ability to self-propagate and transmit its conformation to other proteins. Prions were initially identified as the causative agent in animal TSEs such as
bovine spongiform encephalopathy (BSE)—known popularly as "mad cow disease"—and
scrapie in sheep. Human prion diseases include
Creutzfeldt-Jakob Disease (CJD) and its variant (vCJD),
Gerstmann–Sträussler–Scheinker syndrome,
Fatal Familial Insomnia, and
kuru. A recent study concluded that
multiple system atrophy (MSA), a rare human neurodegenerative disease, is caused by a misfolded version of a protein called alpha-synuclein, and is therefore also classifiable as a prion disease. Several yeast proteins have been identified as having prionogenic properties as well.