Understanding more about how the cell’s batteries are affected in MND

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PhD student Emma Smith has recently started the second year of her MND Association-funded research project at the Sheffield Institute for Translational Neuroscience (SiTRAN) in Sheffield (our project reference: 870-792). With her supervisors Dr Kurt De Vos and Dr Andrew Grierson she is investigating the role of mitochondria in C9orf72-related MND.

basic cell illustration - E Coulthard
Mitochondria (the brown and orange kidney bean shaped structures shown above) provide the cell with energy. Illustration by Emma Coulthard

Mitochondria are the cell’s batteries, providing them with energy. Earlier research has linked damage to mitochondria as a contributor to why motor neurones die in MND. Based on preliminary evidence, the team are aiming to find how the C9orf72 protein causes damage to the mitochondria, where it happens and what might be done to prevent it.

They’ve recently submitted their first year progress report to the MND Association. The project is on track and they’re learning how mitochondria become damaged in MND. One thing that they’ve found is that C9orf72 affects the shape of the mitochondria. If there’s too much C9orf72 protein mitochondria are cleaved into small fragments and if there’s too little, the mitochondria fuse together in one big network.

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.

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