Early Treatment May Thwart Mad Cow-Type Disease
A number of brain diseases caused by mutant prion proteins, such as mad cow disease or scrapie in sheep, might be averted if normal prion proteins can be depleted, researchers report.
Prion disease, which includes the human form of mad cow disease called variant Creutzfeldt-Jakob disease, is thought to be caused when prion protein in the brain becomes misfolded, and this causes normal prion protein to follow suit. The accumulation of mutant prions is toxic to brain cells, eventually turning the brain to mush.
Now UK researchers report that in mice that have been infected with disease-related prions, early depletion of naturally occurring normal prion protein in neurons leads to repair of sponge-like brain damage.
