Do current models of MND accurately represent the disease?

“The results obtained from this research will be fundamental to our understanding of protein aggregation mechanisms in MND.” – scientific peer reviewer.

A common feature of MND is the “clumping” together of proteins which form toxic aggregates that damage cells. Preventing these aggregates from being formed is one possible way to treat the disease. It has been difficult to study aggregates in cells, but recent advances made in imaging techniques could overcome this problem. This method has been successful for other diseases such as Alzheimer’s disease, but has yet to be fully tested in MND.

Through this Junior Non-Clinical Fellowship based at the University of Cambridge, Dr Dezerae Cox is aiming to use these new imaging techniques to understand more about protein aggregates in MND. Using super-resolution microscopy, she will analyse stem cells grown from people with MND with different MND-causing gene mutations, which will allow her to determine the number and size of the aggregates. Dr Cox will then compare aggregates in the cells to those found in human MND brain samples, to determine which cell model is most realistic. This project will provide more accurate models for studying MND and will help us understand more about the role of aggregates in MND, which could aid in developing new treatments.

This project will cost £155,052 over two years.

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