Contact information

David Gordon

Senior Scientist

Utilising a high throughput screening pipeline to identify drugs promoting survival in ALS motor neurons

My research is focused on using mouse models to understand the mechanisms underlying motor neuron loss in amyotrophic lateral sclerosis (ALS). My ultimate aim is to identify new pathways driving ALS, or drugs that can improve translational outcomes for people living with the disease. I am particularly interested in the role of a protein called TDP-43, in which the presence of mutations drive onset and progression of ALS, as well as the adverse effects of oxidative stress on motor neuron loss.

Most recently we have utilised a cell culture model in which an ALS-associated mutant human TDP-43 is expressed in mouse embryonic stem cells, which can then be programmed to become motor neurons in vitro. Using these cells as a drug-discovery platform in high throughput screens, we have identified several pro-survival drugs, and are currently validating candidate drugs in our mouse and human cell culture models.

Recent publications

Aberrant dynein function promotes TDP-43 aggregation and upregulation of p62 in male mice harboring transgenic human TDP-43.

Christoforidou E. et al, (2023), Amyotrophic lateral sclerosis & frontotemporal degeneration, 1 - 10

An ALS-linked mutation in TDP-43 disrupts normal protein interactions in the motor neuron response to oxidative stress

Feneberg E. et al, (2020), Neurobiology of Disease, 144, 105050 - 105050

Mice Carrying ALS Mutant TDP-43, but Not Mutant FUS, Display In Vivo Defects in Axonal Transport of Signaling Endosomes.

Sleigh JN. et al, (2020), Cell reports, 30, 3655 - 3662.e2

Neuronal over-expression of Oxr1 is protective against ALS-associated mutant TDP-43 mislocalisation in motor neurons and neuromuscular defects in vivo

Williamson MG. et al, (2019), Human Molecular Genetics, 28, 3584 - 3599

Single-copy expression of an amyotrophic lateral sclerosis-linked TDP-43 mutation (M337V) in BAC transgenic mice leads to altered stress granule dynamics and progressive motor dysfunction

Gordon D. et al, (2019), Neurobiology of Disease, 121, 148 - 162

The interactome of human TDP-43 in a cellular model of amyotrophic lateral sclerosis

Feneberg E. et al, (2019), EUROPEAN JOURNAL OF NEUROLOGY, 26, 221 - 222

More publications

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