Dr Emma Scotter
University of Auckland
School of Biological Sciences, and Centre for Brain Research, University of Auckland, New Zealand
Dr. Emma Scotter is Head of the Motor Neuron Disease Lab at the Centre for Brain Research, University of Auckland, and Director of the NZ Motor Neuron Disease Research Network.
She is also a Steering Committee member of the NZ Motor Neuron Disease Patient Registry.
Dr. Scotter’s research team investigates the molecular mechanisms of MND pathogenesis, with a focus on protein quality control and genetic modifiers thereof, using human patient post mortem brain cells and tissues.
While New Zealand’s population is small, Dr. Scotter’s team have shown that our rates of MND are among the highest in the world.
Through collaboration with MND research groups and pharma internationally, Dr. Scotter aims to have New Zealanders included in genetic cohort studies and to enable access for New Zealanders to emerging gene-based therapies.
The blood-brain and blood-spinal cord barriers in amyotrophic lateral sclerosis: lessons from human cells and tissues.
Amyotrophic lateral sclerosis (ALS) is a fatal movement disorder driven by the degeneration of motor neurons. Motor neuron health is influenced by both intrinsic and extrinsic factors; dysfunction of the RNA-binding protein TDP-43 in neurons is directly neurotoxic, while TDP43 protein pathology in microglia, astrocytes and oligodendrocytes can promote them to contribute to neurotoxicity indirectly. Breakdown of the blood-brain and blood-spinal cord barriers also accelerates motor neuron degeneration, by bathing neurons in ordinarily partitioned blood factors. In this talk I will highlight the recent work of our New Zealand MND Research hub exploring whether barrier breakdown in ALS correlates with regions of high TDP-43 protein pathology in tissue. I will also discuss how we culture human brain pericytes, a key cellular component of these barriers, and are testing whether those cells derived from ALS patients exhibit dysfunction or TDP-43 protein pathology. Finally I will touch upon the activities of the New Zealand MND Research hub going forward, including nationwide genetic screening.