Brain cell death in diseased mice could be "switched off": study

LONDON, May 7 (PNA/Xinhua) -- British scientists reported on Sunday that they have identified a major path leading to brain cell death in mice with neurodegenerative disease, and by blocking the pathway, the team could prevent brain cell death and increase survival in the mice. Writing in the journal Nature, the researchers at the University of Leicester, UK reported their study of mice with neurodegeneration caused by prion disease. These mouse models currently provide the best animal representation of human neurodegenerative disorders, including Alzheimer's and Parkinson's. In those diseases, proteins "mis-fold" in a variety of different ways resulting in the build- up of mis-shapen proteins, which activates a natural defense mechanism in cells that would switch off the production of new proteins. That would normally switch back "on" again, but in these mice, the continued build-up of mis-shapen protein keeps the switch turned "off". This is the trigger point leading to brain cell death, as those key proteins essential for nerve cells are not made. In this study, the scientists were able to restore protein production by injecting a protein that blocks the "off" switch of the pathway. The brain cells were protected, protein levels and synaptic transmission (the way in which brain cells signal to each other) were restored and the mice lived longer. Because human diseases such as Alzheimer's and Parkinson's have a similar underlying mechanism, the team's results suggest that treatments focused on this pathway could be protective in a range of neurodegenerative diseases. "What's exciting is the emergence of a common mechanism of brain cell death, across a range of different neurodegenerative disorders, activated by the different mis-folded proteins in each disease," said Professor Giovanna Mallucci who led the team at the University of Leicester. "The fact that, in mice with prion disease, we were able to manipulate this mechanism and protect the brain cells means we may have a way forward in how we treat other disorders." (PNA/Xinhua) FPV/ebp

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