Scientists Discover Cause of Motor Neurone Disease and Dementia


Scientists may have made a breakthrough in the quest to understand the cause of a rare dementia from and a motor neurone disease. They have found out how the FUS protein stays in a jelly-like state as it kills off brain cells.

Researchers from Cambridge and Toronto were responsible for the findings, but they are cautiously optimistic of their findings, which could possibly lead to improved treatments of the conditions one day. Susan Greenfield shares the same sentiment, having been involved in the study of Alzheimer’s disease for many years. She is, after all, quite familiar with situations where findings that had every sign of a breakthrough ended up being just false alarms.

MND or motor neurone disease is a terminal and progressive disease that can damage not only the nerve functions, but also that of the muscles. Also known as ALS, it is a condition that affects about 5,000 British adults at one time where they experience severe damage both in the brain and in the spinal cord.

Meanwhile, frontotemporal dementia is a type of dementia that can cause changes to the personality and the behaviour of a person. It can also cause language difficulties to the patient. Both of the conditions are known to be caused by brain cell death.

The researchers decided to look at the FUS protein, which plays a crucial role in ensuring that nerve cells are working properly. The protein has the ability to change its state between more solid jellies or oily droplets. The study has revealed that for both diseases, FUS stayed trapped in the jellied from. While the FUS is in the jelly phase, the machinery that it contains which has the ability to make proteins remains trapped as well which then causes the cells in the brain to fail and eventually, die.

In the case of motor neurone diseases, the protein can get mutated which causes it to get stuck in the jellied from. For frontotemporal dementia, the problem stems from the other enzymes that are responsible for helping the FUS change its state. In this case, they tend to cause the FUS to stay towards the jelly state.

There is a very good chance that the enzymes may hold the key towards getting both the conditions treated. Still, there are a lot of things that are still unknown. For instance, scientists still have no clue about the process is done which is critical information if they are to make a difference this time around. Once this is uncovered though, it is easy to use the enzymes that are controlling the process to either accelerate or inhibit it.

It is important to note that the system that the FUS protein is involved in is delicately balanced. This is also why finding that elusive treatment for both dementia and MND is not going to be easy. This new discovery was successful through the use of human cells resembling neurons along with neurons from frogs.

Stay up to date with the latest developments on Alzheimer’s and dementia by reading about Susan Greenfield online.

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