Identifying novel neuroprotective mechanisms in motor neurone disease

About the project

Our research focuses on identifying new neuroprotective pathways which can be exploited for the treatment of motor neurone diseases. 

Our primary focus is on the childhood motor neuron disease Spinal Muscular Atrophy, but we are also interested in drawing parallels between adult and childhood forms of motor neurone disease, and identifying neuroprotective strategies which are effective in multiple types of motor neurone disease. 

Current projects include:

  • Investigating motor unit recovery following Smn restoration and determining how delayed Smn restoration affects the recovery of the motor unit
  • Understanding pressures put upon enlarged motor units and developing ways to support them
  • Investigating postnatal heart development in mouse models of SMA
  • Analysis of differentially vulnerable motor neurons to identify novel neuroprotective pathways which can benefit multiple motor neurone diseases
  • Investigating the role of P53 in neuronal and non-neuronal tissues in mouse models of SMA;

Funder(s)

Anatomical Society, Muscular Dystrophy Association, Cure SMA

Publication(s)

Mole AJ., Bell S., Thomson AK., Dissanayake K., Ribchester RR., Murray LM.
The rate of Wallerian degeneration following injury is influenced by postnatal maturity, motor unit specific properties and the presence of underlying pathology in mice.
Journal of Anatomy
2020 Apr 20
Villalón E., Kline RA., Smith CE., Lorson ZC., Osman EY., O’Day S., Murray LM., Lorson CL.
AAV9-Stathmin1 gene delivery improves disease phenotype in an intermediate mouse model of Spinal Muscular Atrophy.
Hum Mol Genet
2019 Nov 15
Courtney NL, Mole A, Thomson A., Murray LM.
Reduced P53 levels ameliorate NMJ loss without affecting motor neuron pathology in a mouse model of SMA.
Cell Death and Disease 10:515.
2019 Jul 04
Kline RA., Dissanayake KN., Hurtado ML., Ahl A., Lamont DJ., Ribchester RR., Court F., Wishart TM., Murray LM.
Altered mitochondrial bioenergetics are responsible for the delay in Wallerian degeneration observed in neonatal mice.
Neurobiology of Disease 130:104496.
2019 Jun 06
Kline RA, Kaifer KA, Osman EY, Carella F, Tiberi A, Ross J, Pennetta G, Lorson CL, Murray LM.
Comparison of independent screens on differentially vulnerable motor neurons reveals alpha-synuclein as a common modifier in motor neuron diseases.
PLoS Genet 13: e1006680.
2017 Mar 31

Primary location

Edinburgh

Principal Investigator

Other people involved

Laura Comley (Postdoc)

Victoria Zimmel (PhD student)

Nithya Nair (PhD student)

Alannah Mole (PhD Student)

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