The Scientist published an article this month reporting on advances made by Stanley Prusiner in understanding — and hopefully treating — prion diseases such as CJD, vCJD, and BSE.
In the article, Prusiner — who won the 1997 Nobel Prize for his work on prions — notes that there is still much that researchers do not understand about prion diseases. “We though the number of cases of the disease [vCJD] would increase two to three times,” Prusiner told scientists at the University of Pennsylvania, “but the number of cases in 2001 was similar to the number in 2000.”
That seems to be due to an oddity with vCJD. Whereas CJD generally affects older people, vCJD seems to affect mainly younger people.
The good news is that Prusiner believes there is enough known about prion diseases to start looking at trials to test therapies for the diseases. Some compounds used to treat other diseases also appear to have the ability to prevent normal prions from degenerating into diseased prions that cause CJD.
Prusiner is currently working to develop a mouse model to test such compounds. His plan appears to involve a drug discovery compound to examine 11,000 compounds that might have some effect on prion disease and then test the promising ones in a mouse model before proceeding to human clinical trials.
Source:
Prion-Disease trials on the horizon?. Jennifer Fisher Wilson, The Scientist, 16[6]:28, Mar. 18, 2002.