Exploring mechanisms behind the onset and progression of MND

Most research on motor neurone disease (MND) has focused on how the disease originates with problems in the brain. But IPAN researchers are now investigating the role of skeletal muscle in the disease. 

Through a Fight MND Impact Grant, Professor Aaron Russell is working to expand understanding of how muscle is involved in the onset and progression of MND.  

Together with his research team, he has developed a pre-clinical mouse model that specifically targets the skeletal muscle.  

“Currently most pre-clinical mouse models represent specific genetic mutations that may be found in humans. While these models are important, they represent only about 10% of the known human MND cases,” Professor Russell explained.  

“Our model will be representative of about 90% of all human cases and will be the first MND mouse model to specifically target the muscle. 

 “This means we can investigate the mechanisms that contribute to MND onset and progression, as well as potential avenues for new therapies and treatments.” 

The new model allows researchers to control when the disease pathology starts and to study its progression over time. It even allows them to reverse the disease, giving them significant control when studying potential disease-modifying mechanisms. 

After inducing the disease from within the muscle, Professor Russell will closely examine not only whether the mouse develops functional MND-like changes, but whether it shows deteriorations in certain areas of the spinal cord and brain.   

If successful, this model will become a valuable means to investigate how to change the course of the disease and for testing new treatments.  

“The creation of this model may fast-track the identification of new drugs that could be used in clinical trials to treat MND, with a focus on skeletal muscle,” Professor Russell said. 


In the photo: 

  • Back row –  L to R:
    • Dr Felicity Dunlop (Senior Research Fellow), Professor Aaron Russell, Mr Saddam Muhammad Ishaq (PhD student), Mr Shuzhao Zhuang (PhD Student)
  • Front row – L to R:
    • Ms Saeedeh Robatjazi (PhD Student), Ms Nadia Hafizi Rastabi (PhD Student), Ms Anushka Podar (PhD Student)