Pooping prions - this is more than interesting

Stanley Prusiner won his Nobel prize in 1997 at age 55 for discovering that protein malformation could be contagious. He called the twisted proteins that could catalyze like malformations prions.

Turns out he's still at work ...

Study Gives Insight Into Spread of Chronic Wasting Disease - NYTimes.com

... Researchers are reporting that they have solved a longstanding mystery about the rapid spread of a fatal brain infection in deer, elk and moose in the Midwest and West.

The infectious agent, which leads to chronic wasting disease, is spread in the feces of infected animals long before they become ill, according to a study published online Wednesday by the journal Nature. The agent is retained in the soil, where it, along with plants, is eaten by other animals, which then become infected.

The finding explains the extremely high rates of transmission among deer, said the study’s lead author, Dr. Stanley B. Prusiner, director of the Institute for Neurodegenerative Diseases at the University of California, San Francisco.

First identified in deer in Colorado in 1967, the disease is now found throughout 14 states and 2 Canadian provinces. It leads to emaciation, staggering and death.

Unlike other animals, Dr. Prusiner said, deer give off the infectious agent, a form of protein called a prion, from lymph tissue in their intestinal linings up to a year before they develop the disease. By contrast, cattle that develop a related disease, mad cow, do not easily shed prions into the environment but accumulate them in their brains and spinal tissues.

There is no evidence to date that humans who hunt, kill and eat deer have developed chronic wasting disease. Nor does the prion that causes it pass naturally to other animal species in the wild....

... In captive herds, up to 90 percent of animals develop the disease, Dr. Prusiner said. In wild herds, a third of animals can be infected...

... prions tended to bind to clay in soil and to persist indefinitely. When deer graze on infected dirt, prions that are tightly bound to clay will persist for long periods in their intestinal regions. So there is no chance chronic wasting disease will be eradicated, he said. Outside the laboratory, nothing can inactivate prions bound to soil. They are also impervious to radiation.

So what's the chance that this is the only instance of fecal-oral Prion disease in all of history?

Right. This is probably as common as dirt.

We're going to be learning a lot more about fecal-oral prion disorders in all animals, including humans. We'll also learn how organisms adapt to an indestructible infectious "agent".

This is a big discovery.