Prion infections build quickly then pause before killing
Infectious proteins that cause brain-wasting conditions like mad cow disease appear to build up in the brain long before initiating the cascade of deterioration that leads to dementia and death, a new study of mice finds.
The findings suggest that other factors besides the misshapen infectious proteins characteristic of prion diseases may control the lethality of the disease. If scientists can determine what those factors are, future treatments may be able to prevent the infectious protein diseases — which include mad cow disease, scrapie in sheep and Creutzfeldt-Jakob disease in people — from progressing to a fatal stage.
“We don’t know what’s going on here, but we do know there’s something interesting,” says John Collinge, director of the United Kingdom Medical Research Council Prion Unit in London, who headed the new study.