Taking the STING out of MND — ScienceDaily

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Melbourne researchers are working towards a potential treatment to slow the progression of motor neuron disease (MND), offering hope to people with this debilitating and incurable illness.

The research team have uncovered how inflammation in MND is triggered. Pinpointing the molecules involved in this pathway could be a first step towards a new treatment for MND.

They found that by blocking an immune sensor called STING, they could dramatically prevent inflammation from MND patient cells, paving the way for a new class of drugs to be developed for people with neurodegenerative disorders, such as MND.

The discovery, published today in Cell, was led by Walter and Eliza Hall Institute researchers Associate Professor Seth Masters and Dr Alan Yu, with colleagues from the University of Melbourne and Hudson Institute.

Halting the inflammatory response

MND is an incurable condition in which the nerve cells controlling the muscles that enable us to move, speak, swallow and breathe, fail to work. One in 10,000 Australians will be diagnosed with MND in their lifetime and the average life expectancy from diagnosis is just two years.

Most people suffering from MND have an accumulation of a protein called TDP-43 within cells of the central nervous system. This build-up is associated with an inflammatory response that precedes major symptoms of MND.

Institute researchers investigated how the disease-causing inflammation is triggered in MND, said Associate Professor Masters. “This unexpectedly identified that an immune sensor called STING is activated downstream of TDP-43. Fortuitously, our team had already studied the role of STING in other inflammatory diseases and are now working out how to block it.”

The team then used new inhibitors — drug-like compounds — to block different components of this inflammatory pathway.

“Using cells from patients with MND that we can turn into motor neurons in a