Amyotrophic Lateral Sclerosis (ALS) is a form of MND that has been studied intensively.
However, despite considerable research effort, we still lack a complete picture of what happens within cells that leads to their death. Consequently no effective treatment exists. One of the main hurdles that researchers must face arises because of the nature of the disease. To help overcome this hurdle, researchers can make use of simple cellular models that allow detailed investigation.
Dr Gourlay and his team have recently developed an effective new ALS model system - budding yeast. The published results from this yeast model suggest that mutations in a gene, called SOD1, which are known to cause familial ALS, prevent cells from being able to regulate their metabolism. As a result of this cells cannot function properly and die.
Researchers used the information obtained in yeast to test whether such metabolic dysfunction also occurs in the motor neurones of a model of ALS. They found that simple, but specific, nutritional supplements could prevent motor neurone death in some models.
This project seeks to fully investigate the nature of metabolic dysfunction using ALS model systems and to take these ideas into human ALS trials. This approach will lead to new and exciting insights into how SOD1 mutations drive defects that underpin ALS.
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Many thanks to the BP Coast to Coast cyclists who raised money for MND Scotland to fund this project. Jon Turnbull from the team said, “BP C2C cyclists are delighted to see the funds that they have raised supporting research into a cure for MND. Our late friend Ian Bowen would have been absolutely thrilled to know this”.
Project title: Determining the role of metabolic dysfunction in SOD1-associated MND. Please see MND Scotland's Animal Testing Policy.
Dr Gourlay | University of Kent