Motor Neurone Disease (MND)

Research

Extensive research into MND is being carried out across the world.  In the UK, the Motor Neurone Disease Association is funding research at several centres relating to various aspects of MDS / ALS including:-

  • Genome sequencing
  • Genetic mistakes and therapy
  • Stem cell technology
  • Biomarkers
  • Animal models
  • Toxic proteins
  • A gene-environment database

 

Research at the University of Nottingham & the Queen’s Medical Centre

With support from the NSG@QMC, the Motor Neurone Disease Association and MND Scotland, a hub of MND research has emerged at the University. Collectively, the focus is on understanding the molecular mechanisms that underlie neuronal demise in MND, with particular focus on the SQSTM1 gene that governs the production of the protein p62 which has a role in enabling cells to remove and recycle unwanted proteins. When the process breaks down the build-up of waste is thought to contribute to some forms of MND and frontotemporal dementia (FTD).

With generous support from the NSG@QMC, researchers at the university have established a state-of-the-art human stem cell culture suite, to begin to model MND-associated p62 gene mutations in patient-derived motor neurons. With the goal of utilizing this human model to more rapidly advance biomarker discovery and therapeutic interventions.

Researchers: Miss Sophie Foggin, Dr Raquel Ribeiro, Dr Daniel Scott, Dr Federico Dajas-Bailador and Professor Robert Layfield.

Pioneering work relating to the protein waste recycling process within neurones, which impacts on other neurodegenerative diseases as well as MND, was undertaken by one of our charity’s founder members, Professor John Mayer, and his colleague Professor James Lowe in the 1980s. Since 2000, the late Dr Lynn Bedford, who was also a member of our Management Committee, had carried out research work relating to the 26S proteasome recycling process and the possibility of gene deletion in motor neurones.

  • Eleanor Wilson – “VentMND” Exploring end of life decision making with patients with Motor Neurone Disease (MND) using home mechanical ventilation: The perspectives of people with MND and families.

Further information links:

http://www.mndassociation.org

http://www.wfnals.org/

https://www.nottingham.ac.uk/research/groups/ncare/current-research/index.aspx