A leap in degenerative disease research has resulted in scientists being able to prevent the death of degenerating brain cells in mice — which has the possibility of leading to treatment that could help those struggling with conditions including Alzheimer’s and Parkinson’s.
British researchers, publishing their report in the journal Nature, aimed their experiments at improving our understanding of neuron death in neurodegenerative disease — poorly understood currently due to the difficulty in researching neuron and cell behavior within the living brain.
The scientists discovered a major pathway that led to death in mice with prion disease — the equivalent of Creutzfeldt-Jakob Disease (CJD) — a currently incurable and fatal condition that causes the brain tissue to develop holes and take on a sponge-like texture in the same manner as Mad Cow disease.
An infectious protein called a prion causes the condition, which are ‘misfolded’ proteins that convert their healthy counterparts and eventually cause a build up of infected tissue, affecting normal brain activity. By blocking it, the scientists could prevent healthy brain cells from dying, and therefore managed to help the mice live longer in their experiments.
These ‘misfolded’, built-up proteins are found in many types of degenerative diseases, including Alzheimer’s and Parkinson’s. As misfolded protein levels rise in the brain, the body responds by trying to shut down the production of new, healthy proteins — furthering the onset of such diseases and limiting the amount of healthy protein the body possesses.
If a way can be found to replicate the findings in the brains of humans, then it may be possible to stave off the effects of these diseases for longer — or potentially in the future stop the conditions in their tracks.
Reuters reports that one expert described the research as “a major breakthrough in understanding what kills neurons”, and by manipulating the ’switch’ that shuts down the manufacture of healthy proteins, the mice were able to live significantly longer.
Leader of the study, Prof Giovanna Mallucci, told the BBC:
“The novelty here is we’re just targeting the protein shut-down, we’re ignoring the prion protein and that’s what makes it potentially relevant across the board. What it gives you is an appealing concept that one pathway and therefore one treatment could have benefits across a range of disorders.
But the idea is in its early stages. We would really need to confirm this concept in other diseases.”
The research is still in its infancy, in the same manner as MIT and Georgia Tech developing new methods to explore pathways and neurons in the living brain. However, if both extend to human trials, then potentially we may see breakthroughs in the treatment of neurodegenerative diseases that plague millions of people worldwide.
Image credit: Michael Swan
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