Protecting the nerve cells in motor neurone disease | Action Medical Research

Touching Lives - June 2007

Protecting the nerve cells in motor neurone disease

A good example is the support recently given to a team of scientists looking at the unique properties of a small protein molecule, which could be a key to finding treatments for a range of disabling neurodegenerative diseases.

Work undertaken by a team from Imperial College at Charing Cross Hospital and Birkbeck College in London has shown that a molecule known as heat shock protein 27 (HSP27) can be used to protect motor neurones and help improve damaged muscle function in a laboratory setting.

The study, generously funded by Barnwood House Trust, focused on a condition called amyotrophic lateral sclerosis, but it could impact many conditions classed as motor neurone diseases — progressive disorders of the central nervous system where cells degenerate and die.

Action Medical Research awarded £99,278 to the team in November 2003, allowing them to build on work already done into the protective properties of the protein molecule. Its protective role in models of epilepsy and stroke has already been investigated and recent progress has been good, with the team able to show that increasing the content of HSP27 in a spinal cord model can increase the number of motor neurones and improve muscle function.


Professor Jackie de Belleroche has led the study. She said: “There is still much to be done but we now have strong evidence to show that HSP27 can protect and rescue motor neurones and can provide long-term improvement in muscle function, which has important implications for the treatment of conditions such as motor neurone disease.

“But the benefits could go further. Potentially HSP27 could be used in patients with disabling conditions such as neuropathy and nerve injury, where muscle strength has been impaired.”

No cure

Motor neurone disease actually describes a group of related conditions affecting the motor neurones in the brain and spinal cord, the nerve cells that pass messages to muscles in the body.There is currently no cure.There are around 5,000 people affected by motor neurone disease in the UK and it causes difficulties with swallowing, slurring of speech and shortness of breath.

For most people who develop it, motor neurone disease appears suddenly and for no apparent reason, though it has been established that it can run in some families (less than ten per cent of cases). When the motor neurones in the spinal cord begin to degenerate, the patient suffers a gradual decline and usually dies prematurely as a result of breathing difficulties.

Vital support

Professor de Belleroche says,”We have made good progress but none of this would have happened without Action Medical Research. It is a constant battle to find money to fund work like this, which is perhaps considered less ‘mainstream’ than other areas. I’m glad that Action Medical Research and its supporters recognise just how important it is, because without them, none of this would be possible.

“Thanks to the grant from Action Medical Research we have made some important progress and are now sharing our findings with the wider scientific and medical community through our papers.”

The protective properties of HSP27 could, in the future, lead to the development of therapies to combat the effects of motor neurone disease, perhaps delaying its onset or even reversing the muscle weakness it causes. Human trials are still some way off, but the team’s laboratory research may be an important step that finally leads to a successful treatment for a condition that is devastating both for patients and for their families. TL

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