Role of axonal transport in cell models carrying MND mutations

To establish whether axonal transport deficits in fast motor neurons are a general feature of MND, by comparing different MND models, including rodent and human motor neurons.

Andrew Tosilini

Lead Investigator: Dr Andrew Tosolini

Lead Institution: University College London

MND Association Funding: £142,423* - Junior Non-Clinical Fellowship

Funding dates: November 2021 - October 2023

*Supported by The Lady Edith Wolfson Fellowship Programme

About the project

Healthy motor neurons work by transmitting signals from the brain and spinal cord to muscles to allow us to move, speak and breathe. This relies on a complicated process known as ‘axonal transport’, which refers to the movement of important molecules and organelles (subunits of a cell that have specific functions) up and down motor neuron cells. When people have MND, this transport process doesn’t work as well so the molecules and organelles that cells normally need to survive get stuck in the wrong places. Dr Tosolini’s research, which uses motor neurons developed from people with MND or from mice, will focus on understanding this process, and how it might be restored to normal.

What does this mean for people living with MND?

This project aims to discover novel changes occurring in MND that may contribute to the development and progression of the disease. Through the use of both human cell models and mouse models of MND, the researchers are able to see how axonal transport may change in three different genetic forms of the disease and establish if there are common disruptions in the process between these. The project may help to reveal new targets for future therapies as any deficits found in axonal transport could be potentially be reversed to restore the process.

Resources 

Want to find out more about this project and researcher? Check out the resources below: 

Website:

Our Fellows | MND Association

Blog:

Travel disruptions: transport along neurones in MND 

The awarding of the Junior Non-Clinical Fellowship has enabled me to continue researching MND and drive scientific discovery forwards. I am extremely grateful for the opportunity that the MNDA has given me to continue to drive MND research

Project code: 973-799