Rebecca Brown

University of Wollongong, NSW
Rotary Club of Hunters Hill
Lloyd Binet PhD Scholarship
Motor Neuron Disease 2011

Rebecca Brown is a PhD candidate with the Illawarra Health and Medical Research Institute at the University of Wollongong. She completed a Bachelor of Science (Honours) degree with a major in Biological Sciences in 2010. During her honours year she authored a research thesis which investigated possible interactions between extracellular chaperones and protein aggregates.
 
After thoroughly enjoying her honours year and obtaining a first class result she was encouraged to undertake a PhD study with Professor Mark Wilson investigating motor neuron disease. In 2010 she was awarded an Australian Rotary Health/Rotary Club of hunters Hill Funding Partner Scholarship to investigate the role of protein inclusions in the transfer of pathology in motor neuron disease.  
 
Outside of her academic pursuits, Rebecca enjoys supporting local theatre and performing arts, having performed with the Wollongong Community Orchestra and the Arcadians Theatre Group.


SUMMARY OF PROJECT:
The role of protein aggregation and chaperones in the pathology of MND

Motor neurone disease (MND, also known as amyotrophic lateral sclerosis, ALS) is a serious disease that leads to progressive loss of locomotor abilities, eventually resulting in paralysis and death. There are familial (inherited) and sporadic forms of the disease which may occur at any stage of life and commonly result in death within 6-12 months of the first onset of symptoms. There are currently no treatments for MND. When proteins aggregate inappropriately, they can become toxic to cells and cause nerve damage. Insoluble protein deposits are found inside the nerve cells of MND patients. This and other recent evidence indicates that the inappropriate aggregation of cellular proteins underpin the onset and progression of MND, however the identity of the most important proteins involved in causing the disease is currently unknown. Chaperones are a group of proteins which are able to inhibit inappropriate protein aggregation and in some cases facilitate the refolding of damaged misfolded proteins. Thus, it is expected that chaperones will exert a critical influence on an individual’s susceptibility to MND. Despite this, almost nothing is yet known of the roles that chaperones play in (normally) controlling this pernicious disease.
 
The larger project (of which this PhD is a part) pools the expertise of 6 Chief Investigators (see Reasons for selection of this project, at the end of section 5.), based in both Australia and the UK, to examine the roles of protein aggregation and chaperones in the onset and development of MND in a variety of systems. This PhD project will focus on (i) identifying novel proteins in the deposits found inside nerve cells in MND patients, (ii) studying the aggregation of MND-relevant proteins both in vitro and in cultured cell models, and (iii) using an animal model of MND in which to test the effects of chaperones on disease pathology.
 
This project will provide significant insights into the mechanisms by which protein aggregates and chaperones influence the development of MND. The expected research outcomes will provide a quantum leap in our understanding of those processes that impact upon the development and potential treatment of MND and thereby provide vital clues to possible new directions for therapies to combat MND. Thus, the outcomes of this work address processes of fundamental importance to MND sufferers and their families. Furthermore, the research proposed is highly novel and thus the findings will stimulate a high level of general interest resulting in publications in high impact, international scientific journals for others to access.