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Prions

In 1982, Dr. Stanley Prusiner coined the term “prion” (short for proteinaceous infectious particle and pronounced pree-on) to refer to protein agents that were found in the brains of both humans and animals and that were unusually capable of becoming fatally and untreatably infectious and contagious. Although the destructive nature of these proteins had been witnessed already as far back as in the 1700’s with the sheep disease called scrapie, and then again in the human disease known as kuru by the Fore tribe of New Guinea in the 1950’s, prions did not garner worldwide attention until they affected British cows in the 1990’s and started the highly feared bovine spongiform encephalopathy, or mad cow disease.

The terrifying aspect of this illness that was killing thousands of cows across Britain by attacking their prions and turning their brains into spongelike matter was that it was transmittable to the humans who had eaten the contaminated meat. Also, infected tissue remained highly contagious after death and subsequent boiling, presence in acid, alcohol, or even immersion for years in formaldehyde.

Prions are a normal part of the brain’s makeup, and only become problematic when they change shape from curled to unfurled and set off a chain of unfurling in the other prions in the brain.

Web Resources On Prions

Prions: Killer Proteins
Wikipedia: Prions

Book Resources On Prions

The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases by Philip Yam
Prion Biology and Diseases by Stanley B. Prusiner

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