Taking the STING out of MND

Taking the STING out of MND

Illuminate newsletter index page, summer-2020-21
December 2020

Associate Professor Seth Masters
Associate Professor Seth Masters hopes his discovery leads
to much-needed new treatments for people diagnosed with MND.

WEHI researchers have made a breakthrough discovery that could slow the progression of motor neurone disease (MND). The discovery could lead to new treatments that may add up to 10 years of life for people with MND.

In people with motor neurone disease (MND), nerve cells that control the muscles that we need to move, speak, swallow and breathe, fail to work. One in 10,000 Australians will be diagnosed with MND and the average life expectancy from diagnosis is just two years.

There are currently no treatments to cure or slow the progression of MND.

Curbing inflammation offers new hope

A research team led by Associate Professor Seth Masters and Dr Alan Yu, with colleagues from the University of Melbourne and Hudson Institute of Medical Research, uncovered how inflammation in MND is triggered. 

Associate Professor Masters said it was the first step towards a new, life-changing treatment.

“In people with MND, we see an accumulation of a protein called TDP-43, which sets off an inflammatory immune response that precedes major symptoms of the disease,” he said.

“We unexpectedly identified that an immune sensor called STING is activated downstream of the TDP-43 protein,” he said.

“Using drug-like compounds called ‘inhibitors’, we showed that blocking STING caused a dramatic decrease in inflammation and kept nerve cells alive for longer.”

Associate Professor Masters said the discovery offered new hope for people diagnosed with this devastating disease.

“Although the anti-inflammatory drugs that inhibit STING did not prevent disease onset, they did slow the degenerative progression of disease. We are hopeful this research could lead to a treatment for people with established MND, who currently have very few treatment options and a life expectancy post-diagnosis of just two to five years,” he said.

Dementia and Parkinson's patients may benefit

He said a future treatment might also be effective in slowing the progression of other neurodegenerative disorders, such as dementia and Parkinson’s disease.

“While it is unlikely to be a cure, we hope that future drugs based on this discovery might extend life expectancy and dramatically improve the quality of life for people diagnosed with MND.”

Support needed

To join us in supporting MND research, visit: www.wehi.edu.au/donate

Super Content: 
Two male researchers standing in a laboratory

Our researchers have revealed how a key protein protects against the death of neurons that occurs in Parkinson's disease.

Professor Doug Hilton and Professor Christine Kilpatrick

The grant will enable a new research program – led by researchers at the Walter and Eliza Hall Institute and The Royal Melbourne Hospital – to develop diagnostic tests for the early detection of dementia in people as young as 40.

Animation still showing cells changing

Our biomedical animation team explains the discoveries made by scientists through 3D animation.