Identifying novel neuroprotective mechanisms in motor neuron disease
About the project
Our research focuses on understanding the degeneration and regeneration of peripheral axons and synapses, and identifying strategies to protect motor neurons and encourage regeneration.
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 neuron disease, and identifying neuroprotective and neuroregenerative strategies which are effective in multiple types of motor neuron 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 novel approaches to encourage regeneration following administration of approved drugs in SMA
- Identifying novel mechanisms involved in neuromuscular junction regeneration.
Funder(s)
Anatomical Society, Muscular Dystrophy Association, Cure SMA, SMA Foundation, Muscular Dystrophy UK
Publication(s)
Comley, L.H., Kline, R.A., Thomson, A.K., Woschitz, V., Landeros, E.V., Osman, E.Y., Lorson, C.L. and Murray, L.M.
Motor Unit Recovery Following Smn Restoration in Mouse Models of Spinal Muscular Atrophy.
Hum Mol Genet, 31:3107 (2022)
DOI: 10.1093/hmg/ddac097
Woschitz V, Mei I, Hedlund E, Murray LM
Mouse models of SMA show divergent patterns of neuronal vulnerability and resilience.
Skelet Muscle. 12:22 (2022)
Mole, A.J., Bell, S., Thomson, A.K., Dissanayake, K.N., Ribchester, R.R. and Murray, L.M.
Synaptic withdrawal following nerve injury is influenced by postnatal maturity, muscle-specific properties, and the presence of underlying pathology in mice.
J Anat, 237, 263-274 (2020)
DOI: 10.1111/joa.13187
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. Nov 15;28(22):3742-3754 (2019)
DOI: 10.1093/hmg/ddz188
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)
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)
2023 Sep 26Primary location
Edinburgh
Principal Investigator
Other people involved
Laura Comley (Postdoc)
Nithya Nair (PhD student)
Inga Partlova (PhD Student)