There is hope of a breakthrough in treating motor neurone disease (MND) after Edinburgh researchers found a way to repair nerve cells damaged by the condition.
And existing drugs could be part of a new treatment.
The New Daily reports that the new technique amounts to “boosting the energy output from the mitochondria, the cells’ power supply”.
This could mean using existing drugs that boost mitochondrial function and would be repurposed to treat MND.
One candidate is a licensed diabetes medication.
The team at the Euan MacDonald Centre for MND Research at Edinburgh University has shown that the “axon – a nerve fibre, sometimes up to a metre long, which connects and sends electrical impulses from the nerve cells to the muscle – is shorter in cells affected by MND than in healthy cells,” Scotland’s Herald reported.
“They also discovered that the movement of the mitochondria, which travel up and down the axons, is impaired.
“However, the researchers found that the damage to nerve cells – or motor neurones – caused by MND can be repaired by boosting the energy levels in these mitochondria, the tiny ‘batteries’ which power chemical reactions in all human cells.
“Once this was done, the axon reverted to normal length.”
Research lead Dr Arpan Mehta, a trainee neurologist, says the results are “really exciting”.
“We believe that this technique of rescuing the axon through boosting energy will not only slow and stop but could reverse the degeneration, so it could potentially be very powerful.
“The importance of the axon in motor nerve cells cannot be understated.
“Our data provides hope that by restoring the cell’s energy source we can protect the axons and their connection to muscle from degeneration.
“Work is already under way to identify existing licensed drugs that can boost the mitochondria and repair the motor neurons. This will then pave the way to test them in clinical trials.”
The MND-SMART project, run by the McDonald Centre, is an “adaptive clinical trial”. By bringing together hundreds of patients across the UK it enables multiple possible treatments to be tested at once.
“If you were to hand a drug that’s already safe – because it’s used in other diseases – to MND-SMART, you could basically get a result within a couple of years,” said Dr Mehta.
“Because the stark reality is, despite all the progress in modern medicine, the only drug that is licensed for this condition is riluzole, which was licensed in the mid-1990s and prolongs life by two to three months in a condition with average survival of two to three years.”
MND affects the nerves (motor neurones) that communicate between the brain and the muscles that enable us to move, speak, swallow, and breathe. In people with MND, the motor neurones gradually degenerate and die, causing the muscles to weaken and waste.
MND is a life-limiting disease and, although MND progresses differently for each person, the average life expectancy is two to three years.
UK charities hailed the study, including the foundation set up by Scots rugby legend Doddie Weir.
In Australia, former AFL star and coach Neale Daniher has been raising awareness of MND since being diagnosed with the illness seven years ago.
Last week, the Victorian government announced a famous walkway to the MCG would be renamed in Mr Daniher’s honour. The walkway from William Barak Bridge to the MCG will be renamed Daniher’s Way.
“This is a small gesture to a man who has made a huge contribution to football, to medical research and Victoria,” said state Premier Daniel Andrews. The government also made a $1 million donation to FightMND, the organisation founded by Mr Daniher.
“We are so, so pleased we have been able to work with the MCC and the trust and with Daniher’s Drive and Neale himself and his family, to appropriately honour someone who continues to work very hard for others,” Mr Andrews said.
FightMND has raised more than $40 million, including $11.9 million in 2020 despite its MCG ‘Big Freeze’ fundraiser being cancelled due to COVID-19.
Should our research bodies adopt the MND-SMART model? What diseases do you think we will see treatments for in our lifetime?
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