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Dr Emma Louise Scotter (nee Daniel)

Dr Emma Scotter

The University of Auckland, Department of Pharmacology, Centre for Brain Research

The Theory of (not quite) Everything: The neglected role of the blood-brain-barrier in motor neuron disease

Dr Emma Scotter is a cell biologist who seeks to understand the mechanisms involved in degenerative brain diseases. Emma obtained her PhD from the University of Auckland, specialising in Huntington’s Disease. In 2010 she was awarded a Marie Curie Fellowship to work at King’s College London, where she shifted her focus to Motor Neurone Disease. She discovered that proteins which forms clumps in patient brain cells can be disposed of by cells under certain conditions, meaning that drugs that activate these disposal processes might be of therapeutic benefit. Emma returned to Auckland in 2014 as an Aotearoa Fellow at the Centre for Brain Research, where she continues to examine protein waste disposal in Motor Neurone Disease. She works closely with the Human Brain Bank and Biobank, clinical neurologists, and Motor Neurone Disease care groups to undertake studies using cells and tissue from patient donors and to communicate novel findings to the patient community.

Research

Awareness of motor neuron disease (MND) is growing, following the hugely successful ice-bucket challenge and the release of the Stephen Hawking biopic “The Theory of Everything” in 2014. But we are poised on a precipice; increased awareness and research funding must yet be translated into understanding of disease mechanisms before patients can benefit from better treatments. Indeed, the typical sufferer of MND will die from the disease within 3 years, meaning that Hawking currently represents the exception, rather than the rule.

MND is a fatal and incurable movement disorder affecting ~1 in 15,000 New Zealanders. In this disease, motor neurons within the brain and spinal cord degenerate, causing progressive loss of movement function. Both genetic and environmental factors contribute to motor neuron death in MND. But do these factors also affect other types of brain cells? Dr Scotter has exciting new evidence to suggest that they do. Pericyte cells, which surround the blood vessels in the brain and form part of the blood-brain-barrier, are also damaged in MND. This may explain how blood-borne irritants can leak into the brain in MND, which worsens the plight of the motor neurons.

This work will use pericyte cells grown directly from the brain and spinal cord of MND patients who have bequeathed these tissues to our Human Brain Bank. This incredible resource will allow Dr Scotter’s group to conduct two important studies. The first study asks whether pericytes from brain regions containing dying motor neurons show the same disease signatures as pericytes from brain regions which are spared in MND. The second study then tests a range of chemical compounds for their ability to provoke or alleviate a disease signature in pericytes. Together these studies will determine whether the disease processes occurring within motor neurons also occur independently within pericytes.

This work has important implications for identifying new treatments for disease – by helping Dr Scotter’s group to identify all of the cell types an effective treatment for MND must target, as well as allowing them to test potential new treatments using cells other than just motor neurons. By exploring the role of non-neuronal cell types such as pericytes, they hope to better understand why MND develops and how they can best treat it. And perhaps one day soon they will have their “Theory of Everything” regarding this devastating disease.