Deposits of the protein TDP-43 are found within the motor neurones in the majority of cases of MND, and are considered a pathological hallmark of the disease. While we do not fully understand how these deposits are formed, previous research has shown that activation of a process called the Unfolded Protein Response (UPR) can cause TDP-43 protein to deposit in the motor neurones.
At the University of Bradford, Prof Marcus Rattray and Dr David Hicks are conducting tests to identify which biological pathways link the UPR and the formation of TDP-43 protein deposits within the motor neurones (our grant reference: 837-791).
Once the right connecting pathways are found, the team will then test whether they can target them with drugs to reverse the accumulation of TDP-43, which may have an effect on slowing the progression of MND. The researchers will screen hundreds of compounds that may potentially block or reverse TDP-43 accumulation. As they are testing compounds that are already safe for use in people, any that block or clear TDP-43 deposits have huge potential to be fast tracked to a clinical trial.
Throughout June 2016 MND Awareness Month will be highlighting the rapid progression of the disease in its powerful Shortened Stories campaign, sharing the experiences of people currently living with MND, or who have lost loved ones to the disease, through art, poetry and film.