A chemical substance has been identified which shows signs of being able to halt a range of neurodegenerative diseases in mice.

Researchers at the University of Leicester’s Medical Research Council Toxicology Unit, investigating prion disease in mice, have found a common feature across all types of brain cell death. Looking at the natural defence mechanisms of brain cells, they found that brain cells respond by shutting down protein production when attacked by a virus (in order to halt the virus’s spread).

A range of neurodegenerative diseases involve the formation of faulty or "misfolded" proteins, and these activate similar defence mechanisms. In Parkinson’s Disease it is alpha-synuclein which is the errant protein, in Alzheimer’s Disease it is amyloid and tau, and in the case of Huntingdon’s Disease it is another different protein.

The researchers believe the specific errant protein is irrelevant, because it is how cells deal with misfolded protein which is important. The presence of misfolded proteins causes brain cells to shut down protein production for a long period, eventually resulting in cell death.

The researchers believe they may be able to disrupt this process by the administration of a particular compound, thereby halting neurodegeneration; at least in mice with prion disease so far.



DOI: 10.1126/scitranslmed.3006767

October 14, 2013