British researchers report that they have found a major pathway leading to brain cell death in mice with prion disease, the mouse equivalent of Creutzfeld-Jacob Disease (CJD). They then worked out how to block it, and were able to prevent brain cells from dying, helping the mice live longer.

The finding points to a common mechanism by which brain diseases such as Alzheimer's, Parkinson's and CJD damage the nerve cells.

In neurodegenerative diseases, proteins "mis-fold", leading to a build up of misshapen proteins, the researchers explained. These misshapen proteins form the plaques found in the brains of patients with Alzheimer's and the Lewy bodies found in Parkinson's disease.

Giovanna Mallucci and colleagues found that the build up of mis-folded proteins in the brains of mice with prion disease activated a natural defense mechanism in cells, which switches off the production of new proteins. This would normally switch back on again, the researchers explained, but in these ill mice the continued build-up of misshapen proteins keeps the switch turned off. This is the trigger point leading to brain cell death, because key proteins essential for cell survival are not made.

By injecting a protein that blocks the "off" switch, the scientists were able to restore the production of the survival proteins and halt the neurodegeneration.

Eric Karran, director of research at Alzheimer's Research UK, said while the research was still at an early stage, the results were exciting.

"While neurodegenerative diseases can have many different triggers, this study suggests that they may act through a common mechanism to damage nerve cells. The findings present the appealing concept that one treatment could have benefits for a range of different diseases," he said.

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