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Spotlight on research: Tracking proteins to understand how MND spreads

Science in brief
In a world-first demonstration, the researchers were able to track the movement of disease proteins in real time and show their uptake into neuroprotective cells known as microglia, which had been attracted towards the dying neurones. This was the first time this process has been visualised in a living animal. Moreover, the researchers were able to show the spread of these disease proteins into the surrounding tissue and that this process was significantly altered when microglia were absent.

Investigating whether aggregated proteins are released from a stressed or dying motor neurone in a living experimental model will help researchers understand the role these proteins play in causing familial and sporadic MND. The zebrafish is a useful model as these visualisation studies cannot be tackled in rodents or humans. Importantly, this model will become an excellent tool to evaluate potential therapies aimed at blocking the spread of neurone death.

Next steps
Real-time characterisation of proteins in cells establishes a strong foundation for future funding applications for the next stages of this research. The spread and transfer of MND aggregates may be a common mechanism for both sporadic and familial MND and is therefore important for all MND cases. Importantly, if these neuroprotective cells are responsible for controlling the spread of neurodegeneration, this would uncover new targets for disease-modifying therapies.

Article sourced from MND Research Institure of Australia report '$25Million, 25 Milestones: Changing the future of MND'. 


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