Stopping the Progression of Motor Neurone Disease

New research from the University of Sheffield's Institute for Translational Neuroscience (SITraN) looked at how ways of slowing the progression of motor neurone disease (MND).

This incurable disease that works by destroying the body's cells and in turn, prevents movement as the problem progresses, has no cure and a relatively limited amount of treatments. Yet researchers looked at the onset of symptoms that caused the progression of the disease and the prescense of a known genetic cause of the condition.

Researchers investigaged two mouse models of MDN caused by alteration in the SOD1 gene, with the known cause of MND in humans. One of the strains that's rapidly progressing while other showed symptoms at a much slower rate with MND. The teams looked at the various factors that might cause such differences via speed and severity in the progression of the disease. They used a scientific technique that's known as gene expression profiling in order to identify various factors regarding neurones that control vulnerability or resistance of MND in order to shet light on various factors that are important to speed of motor neurone injury in human patients.

Study results showed that at the onset of the disease, mucles weakness tends to be observed---which shows a key difference in major molecular pathways and the way the protective systems of the body respond. For instance, in the model of those with the rapidly progressing MND, the motor neurones showed reduce functioning fo the cellular systems for energy production, disposal of waste proteins and neuroproduction. For the others, ohwever, MND showed an increase in protective inflammation and immune resonses and increased function of the mechanisms that protect motor neurones from damage.

Researchers hope this new information can provide valuable clues regarding the mechanisms that effect the slowing and progression of these health issues.

"MND is a clinically heterogenous disease with a high variability in its course which makes assessments of potential therapies difficult. Thanks to the recent evidence in our laboratory of a difference in the speed of symptom progression in two MND models carrying the same gene mutation and the successful collaboration with Professor Pamela Shaw and her team, we have identified some mechanisms that may help to predict the disease duration and eventually to slow it down," said Dr Caterina Bendotti, via a press release. "I strongly believe that the new hypotheses generated by this study and our ongoing collaboration are the prerequisites to be able to fight this disease."

More information regarding the study can be found via the scientific journal Brain.