http://www.nature.com/news/2008/080904/full/news.2008.1080.htmlTest tube experiments may help identify the most hazardous prion proteins.
Amber Dance
Mixing up normal and infectious prions in test tubes can generate entirely new forms of infectious prion.GettyInfectious prion proteins from hamsters can change normal proteins from mice into new, infectious forms of prion - simply by mixing the proteins together in a test tube.
Researchers at the University of Texas Medical Branch in Galveston suggest their discovery could be turned into a useful test for whether a given prion strain is transmissible from one species to another. Prion proteins are responsible for Creutzfeld-Jakob disease and "mad cow" disease.
But they also found that when a prion jumps species, it produces a new kind of prion. "This is very worrisome," says Claudio Soto, who led the research, published in Cell1. "The universe of possible prions could be much larger than we thought."
Normal prion protein, or PrP, is found throughout the body but is concentrated in the brain. Its exact role is not known, although it has been linked to cell signalling2, metal-ion transport3, and blood-cell manufacture4. The protein can adopt malformed shapes that cause disease. Those proteins, which are resistant to degradation, bind and convert normal protein to their troublesome conformation. Over time, the diseased protein builds up and forms fibrils in the brain, causing neurodegeneration and ultimately death.
Generally, prions are limited to a specific host and a few related species. But prions sometimes cross the species barrier to infect new hosts. Notably, prions from cows have hopped to humans, causing disease in 208 people, mostly in the UK. Now, scientists wonder if the prion-induced chronic wasting disease (CWD), which afflicts elk and deer in the US, could jump to humans. Since prion diseases have long dormant periods, the fact that there are no human cases of CWD doesn't necessarily indicate that people won't develop symptoms in the future.
Jump the barrier