A/Professor Stephanie Brown

Motor Neuron Disease Research

‘Modelling sporadic Amyotrophic Lateral Sclerosis (ALS) using induced pluripotent stem cells derived from ALS patients’

University of Wollongong, NSW
Awarded 2014-2016

Co-Funded by Rotary Club of Cronulla, NSW

“The motor neurons will be studied to understand changes in cellular stress, which we hope will aid in understanding disease pathways and provide novel therapeutic strategies for the disease.”

Researcher Profile

Born in Nowra, Monique Bax grew up in Hamilton, New Zealand. Monique completed high school at Hamilton Girls’ High School, were in her final year she was the President of the Student Council, member of the Academic Council, debate team, cultural committee and was awarded the fellowship cup and prize by her peers.

Following high school, Monique deferred her offer of admission to the University of Wollongong, taking a year off study to work at a law firm and travel in Europe.   During this time she came to realise her love of science, and consequently moved back to Australia, and began her degree at the University of Wollongong. Monique is currently in the fourth year of a Bachelor of Medical Biotechnology with Honours. Throughout her degree Monique has been engaged in many fascinating areas of science including anatomy and physiology, organic and analytical chemistry, biochemistry, biomedical physics, biotechnology, genetics, pharmacology and immunology.   These subjects have helped prepare Monique for the current research she is undertaking. Monique has recently completed an Honours research project, examining changes in lipids during induced pluripotent stem cell differentiation relevant to neurodegenerative disease, supervised by Dr. Lezanne Ooi and Associate Professor Todd Mitchell. Throughout this project Monique developed a strong interest in understanding the mechanisms behind neurodegenerative diseases and wishes to continue research in this area in future.

Project Summary

Aims:

  1. Develop a new, non-invasive method to generate and culture motor neurons from Motor Neurone Disease patients using induced pluripotent stem cells.
  1. Use these motor neurons to understand changes in Motor Neuron Disease cells that lead to neuronal death and disease.

Summary:

We will be reprogramming skin cells from ALS patients into stem cells (induced pluripotent stem cells). This will be achieved using a new method, to change the gene expression in the cells while maintaining the original genetic sequence of the donor. These cells will be characterized, examining the protein and gene expression of the pluripotent stem cells.

These induced pluripotent stem cells can then be converted into motor neurons using a defined set of growth factors. The motor neurons will be studied to understand changes in cellular stress, which we hope will aid in understanding disease pathways and provide novel therapeutic strategies for the disease.

Supervisors: Dr Lezanne Ooi & Dr Justin Yerbury