Prion

(noun. PREE-ahn) 

by Olivia Conway

What does it mean? 

A prion is a misfolded protein, typically found in the brain, that can induce other proteins around it to misfold as well. Misfolding means that these proteins do not have the structure they need to function properly, so they can group together and form aggregates. These clumps of misfolded proteins can lead to serious neurodegenerative diseases that are transmitted by ingesting brain matter contaminated with the prions. Scientists still don’t understand what causes normal proteins to misfold into prions. The conditions associated with prions, which also occur in other mammals, are currently untreatable. 

How do I use it in a sentence?

Mad cow disease, or bovine spongiform encephalopathy, is caused by a prion.” 

The left image shows a normal protein structure that will not cause illness. The right image shows the structural changes to the protein that cause disease. The change from the sheet form to the beta helix form (red) can cause structural changes in other proteins and toxic aggregates that are responsible for neurodegenerative diseases. Image source 

History of usage

The word prion was developed in 1982 by biochemist and neurologist Dr. Stanley Prusiner from the words protein and infection to describe the phenomenon of an illness transmitted by the misfolded proteins.

Fields of study in which this word is commonly used

Biochemistry, cell biology, neuroscience, molecular biology, medicine

Edited by Ena Vujic