The word prion, coined in 1982 by Stanley B. Prusiner, and is
derived from protein and infection. Hence prion means
"proteinaceous
infectious particle", in reference to its ability to self-propagate and
transmit its conformation to other proteins.
A
prion is an infectious agent
composed entirely of protein material, called PrP (short
for prion
protein), that can fold in multiple, structurally and distinct ways, at least one of which is
transmissible to other prion
proteins, leading to disease that is similar to viral infection. They are
suspected to be the cause of transmissible spongiform encephalopathies (TSEs)
among other diseases.
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-Straussler-Scheinker syndrome, fatal familial insomnia, and kuru. A 2015
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.
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