Finding a connection between the cell’s waste disposal system and MND

Just like when we put out our recycling every week, the cells in our body have their own recycling system too. One of the recycling plants within our motor neurones works by a system called autophagy. In a recently completed research project that the MND Association has funded Dr Rob Layfield and Dr Alice Goode have been looking at how malfunctions in autophagy cause MND (our reference 821-791).

Their research has focussed on how a gene called SQSTM1 and the protein it makes (the protein is known as ‘p62’) is involved in MND. The project has gone well and we have a much better understanding of how cell recycling goes wrong in MND.

Dr Alice Goode

Dr Alice Goode

One finding was that where the mistake was in the gene had quite different and specific consequences. The results of their research into the effects of one specific mistake have recently been published in the journal Autophagy (Goode et al Autophagy 2016, 12(7), 1–11).

A mistake in a gene can lead to the creation of a protein with a slightly different shape to normal. Dr Goode and Dr Layfield found that the change in shape in the p62 protein meant that it was less likely to hook into the correct part of the recycling plant machinery. If you’ve ever watched the way a recycling bin is hooked into the back of a lorry and tipped up to be emptied, this mistake is like the hook being slightly the wrong shape.

Dr Rob Layfield

Dr Rob Layfield

As well as working out that the hook was faulty, the researchers also showed that this meant that it was three times less likely to hook up properly than proteins that were made correctly.

We’ve recently agreed to fund a follow on research grant to continue this work.

This new project intends to build on the achievements so far, importantly including the use of models we have developed to try and identify existing autophagy-enhancing drugs that might be re-purposed for the treatment of MND” explained Dr Layfield recently.

 

 

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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