How Stanley Prusiner Discovered Prions and Won a Nobel Prize

Thursday, May 22, 2014

Prion Protein Fibrils Prion Protein Fibrils (AJ Cann/NIAID/flickr)

In 1997, Stanley B. Prusiner received a Nobel Prize for identifying the agent responsible for ravaging the brains of animals suffering from scrapie and mad cow disease, and of humans with Creutzfeldt-Jakob disease. His investigation had been waged largely alone and, in some cases, in the face of strenuous disagreement. In Madness and Memory: The Discovery of Prions—A New Biological Principle of Disease, Prusiner tells the remarkable story of his discovery of prions—infectious proteins that replicate and cause disease but contain no genetic material. He talks about the work of teams of scientists who persevere in the face of opposition, and looks into what it will take to cure Alzheimer’s, Parkinson’s, Lou Gehrig’s and other devastating diseases of the brain.


Stanley B. Prusiner

Comments [9]

Sredni Vashtar from the Shed Out Back

Great interview on a fascinating topic. One quibble: While I appreciate the point being made by calling cows "cannibalistic" I think the sensationalistic "eww" factor detracts from the larger message. Also, framing it that way isn't even accurate. I presume cows are not being fed bovine meat and have therefore not developed a taste for it. One ingredient in their feed just happens to be derived from other cows (and happens to be a vector of BSE transmission).

May. 23 2014 08:09 AM
Anonymous from Uptown

We should absolutely have more funding for this -have been researching Cancer for years and we have not obliterated it yet. why not?

May. 22 2014 12:37 PM
Amy from Manhattan

What are the chances that the disease process can be kept from advancing if prion diseases are diagnosed early enough, & what approaches are most promising?

May. 22 2014 12:37 PM
karen from NYC

My mom died of Creutzfeld Jacob in 1983 when she was 52 years old. She also had a cousin of a similar age who died of the same disease within a few years of that. When she died I did some research at the mid-Manhattan library to find out more about it, and there wasn't much to find. Do you think, as I learned from my research, that the incubation period was 40 years and they possibly ate something at a family gathering when they were teens that caused the disease?

May. 22 2014 12:32 PM
Fred from Queens

How do prions consumed through cannibalism survive the digestive tract?

May. 22 2014 12:32 PM
Michael from nyc

I heard that prions cannot be killed on medical instruments. If that is so, then should instruments used in brain surgery be used only once?

May. 22 2014 12:26 PM
Dan from New York

Dr. Frank Bastian at LSU has evidence of a wall-less bacterium in the eyes of Scrapie sheep and ,in recently published findings, evidence of a form of transmission. Have you considered this evidence in the light of your own theory?

May. 22 2014 12:20 PM
Amy from Manhattan

What's known about the mechanism by which prions induce normal proteins to change their shape to match that of the prions? Are there conditions in the brain (& elsewhere) that put the equivalent of evolutionary pressure on proteins, promoting the existence & propagation of prions over normal ones?

May. 22 2014 12:19 PM
John from Westchester

For air? Thank-you
Dr Prusiner, just two years after you received your Nobel prize, there appeared in popular culture a "mad" character with a spongiform head (SpongeBob SquarePants)... Have you had a chance to talk with those creators to see what they were thinking when they did that?

May. 22 2014 10:42 AM

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