MND is a group of diseases in which the motor neurons (the nerve cells that signal from our brain to tell our muscles to move) become damaged and start to die. This causes our muscles to become weaker and waste away.
Developing treatments for MND has been difficult because it does not have a single cause, there are many genetic and environmental risk factors. Another major challenge is that there is great variability in the presentation of the disease. What underpins this variability is not understood, yet it is critical to developing patient-specific treatments.
Professor Coral Warr of La Trobe Institute for Molecular Science (LIMS), together with Professor Tracey Dickson at University of Tasmania, has been awarded an MND Research Australia Innovator Grant to investigate the variability seen in the presentation of MND.
“We will test the idea that the variability seen in disease presentation is related to the motor neurons becoming overly excitable, meaning they respond too easily to signals,” explained Professor Warr.
“There is evidence that the level of excitability is linked with disease duration and severity, which leads us to hypothesise that the mechanisms that usher this change in excitability are linked to the variability in disease presentation.”
To test this idea the researchers will use the fruitfly Drosophila melanogaster. Flies have very similar motor circuits to humans and are excellent animal models for MND. “By using flies we can study disease progression across ageing, which has been very difficult to do in other models,” said Professor Warr.
Professors Warr and Dickson are hoping their project, using the humble fruitfly, will help map out the events that lead to the loss of ability to keep neuron excitability in check in MND, and how this differs in patients with different clinical features.
This will provide important information regarding potential intervention points and therapeutic targets in different patients.
Professor Coral Warr
Professor Coral Warr’s lab in LIMS studies how cells in the nervous system receive and respond to signals from the environment. They use the fruit fly Drosophila melanogaster as a model organism as many of the major cell signalling pathways in mammals are conserved (and were in fact first discovered) in flies. Signalling between cells in the nervous system is highly conserved between flies and humans, and there are highly sophisticated genetic and molecular approaches available to study gene function and expression.
MND Australia is committed to advancing research and care for people with MND. Professor Warr received the 2022 Innovator Superball XIV MND Research Grant from MND Research Australia.