Latest research news from the clinic and the labs

The Euan MacDonald Centre Investigators continually publish their findings in high-quality peer-reviewed academic journals, and the last six months have been no exception.

Of the approximately 80 papers published since the Summer this year, there were 38 that have particular relevance to MND, related neurodegenerative conditions or the biology of motor neurons.

The more scientifically minded among you can view the full list here: Euan MacDonald Centre publications Jul-Dec 2014 [PDF, 143KB]

Six steps are needed to cause MND

robert swinglerShuna ColvilleSome of our supporters may have contributed to the  Scottish MND Register. You will be pleased to know that data from the Register were included in a recent study, led by Ammar Al-Chalabi of King’s College London and Neil Pearce of the London School of Hygiene and Tropical Medicine, which was published in the prestigious journal, Lancet Neurology. Euan MacDonald Centre Investigator Dr Robert Swingler and research nurse Shuna Colville (who is also Manager of the Anne Rowling Regenerative Neurology Clinic) were authors on the study. In this study, the researchers analysed the age of onset in 6274 cases of ALS from five registers in the UK and Europe. Using a mathematical model, the team concluded that ALS is a multi-step process that is likely to require six different steps. In other words, six different genetic, lifestyle and environmental factors are needed to trigger the condition.

You can read more about this study, and an interesting parallel with cancer, in a blog on the MND Association’s research blog: Lessons learnt from cancer – identifying the causes of MND

Fibre-optic imaging of muscles to diagnose and monitor MND

Richard_RibchesterProf Richard Ribchester and colleagues published their progress in an effort to combine electrophysiological recording (electromyography; EMG) with a live imaging technique that utilises a slender fibre-optic imaging probe connected to a confocal microscope. They envisage that development of this methodology may eventually be applied to the diagnosis and monitoring of disease progression, and treatment, in patients with neuromuscular disease, including ALS/MND.

Motor neurons and vulnerability to cell stress

Cathy_AbbottProf Cathy Abbott and her former PhD student Lowri Griffiths, with colleagues from the NYU Langone Medical Centre and elsewhere, have uncovered the mechanisms by which cells continue to function normally under conditions of stress. The work provides new insights into why motor neurons might be particularly vulnerable to cellular stress. Read more about this in our news item from September 2014.

Using stem cells towards designing regenerative therapies

dave-lyons_124x180In the zebrafish, nerves can regenerate after damage. Euan MacDonald Centre scientist Dr Dave Lyons is working to understand the nervous system in zebrafish, and what signals permit and drive nerve regeneration. Dr Lyons has published new findings recently, specifically regarding the role of supportive cells called glia in the development of the nervous system.

 

 

 

siddharthan-chandranIn human stem cell studies, Prof Siddharthan Chandran and colleagues are beginning to examine ways to prolong the survival of ALS stem cell-derived nerves grown in a dish. The researchers reported that stimulating a form of self-digestion called autophagy can be useful to clear toxic aggregates of the protein TDP43. Meanwhile, Prof Sir Ian Wilmut is co-author of a report on harmonising standards to produce clinical-grade stem cell therapies.

Measuring cognitive change in people with MND

sharon-abrahams  thomas bak_124x180Prof Sharon Abrahams and Dr Thomas Bak have revealed that some people with MND display problems related to “theory of mind”. This specific difficulty in identifying and distinguishing the feelings and thoughts of another from one’s own may underpin the social behavioural problems that some people with MND experience, and might manifest as apathy and loss of awareness.

In a related study, Prof Abrahams’ PhD student Ratko Radakovic reviewed studies that have used different methods to measure apathy and found that some were not as robust as previously thought. In particular, Ratko concluded that there was no suitable scale with which to measure apathy in MND. Read more about this study on the Anne Rowling Clinic website.

Through collaborators, the team has tested the German/Swiss-German version of the Edinburgh Cognitive ALS Screen (ECAS) and concluded that it is a fast and easy-to-administer test that is sensitive for ALS-specific problems and behaviour change.

Progress towards therapeutics in spinal muscular atrophy

Tom_GillingwaterTom Wishart_124x180Prof Tom Gillingwater, with Dr Tom Wishart and others, has made significant inroads in understanding the childhood motor neurone disease, spinal muscular atrophy. They have recently published findings relating to the molecular mechanisms of this condition and how cells try to compensate for low levels of a protein called SMN. The team is now testing different molecules as potential therapeutics in mouse models.

And in related news….

Because many of the fundamental cellular mechanisms of nerve degeneration are shared among different conditions, research on these related conditions can be highly applicable to MND.

DTara Spires-Jonesr Tara Spires-Jones focuses on Alzheimer’s disease, in particular using a state-of-the-art technique called array tomography to image human brains. The team has noticed alterations in several brain proteins in people with dementia, which may give clues into disease cause and progression. Excitingly, some of these proteins are also beginning to be tested as therapeutics.

 

 

 

don-mahad_124x180Dr Don Mahad focuses on multiple sclerosis (MS). He has published recently that mitochondria, the “powerhouses” of the cell, are likely to play a central role in nerve cell degeneration in MS. This may equally well have implications for MND.

 

Congratulations to all our Investigators and keep up the good work!