Professor Yerbury's Legacy

By Dr Maretta Mann

On 11th October Inclusive Futures: Reimagining Disability held a Book Club event in memory of the late Professor Justin Yerbury, an award-winning research scientist, disabled academic, and author of Fighting Fate.

Justin's wife, Dr Rachel Yerbury opened the event and spoke about discrimination in academia. Members of the Yerbury Group at University of Wollongong, Victoria Shephard, Thomas Walker, and Dr Isabella Lambert-Smith, followed with an update of the team's research.

Professor Justin Yerbury in the laboratory looking at a computer screen with two colleagues

Dr Rachel Yerbury: Disability and academia

"We are here today to remember the work of my husband Professor Justin Yerbury.

Among his many roles Justin was a husband, a father, a research scientist and a disabled academic. He took these aspects of his life very seriously and in each case he put all his efforts into being the best that he could be. And in doing so he set an example for other people in similar situations. ... Other research scientists were motivated by Justin's passion and energy, and by the way he shared, collaborated but never compared. As a disabled academic Justin fought with courage and dignity to find a cure to the disease that he, himself, lived with.

To say he was inspiring was an understatement. Justin showed the world what a completely paralysed person on life support could achieve. But he didn't only fight for himself and against the disease, he relentlessly fought against ableism and discrimination. Justin was well supported by the University of Wollongong who did everything they could to stand with him against ableism and inequity. But in the wider world he saw that this wasn't always so.

As a family we experienced discrimination in lots of ways and places. But what most upset him was the experiences of his colleagues and peers in academia. In 2020, Justin took on the National Health and Medical Research Council (NHMRC) over the fairness of achievement of policies to opportunity. He finally made them see reason and they subsequently changed their policies and criteria in an attempt to level the playing field.

Together Justin and I co-authored a paper in Trends in Neurosciences called "Disabled in academia: to be or not to be, that is the question". The paper highlighted the challenges of academics living with disability and neurodiversity, and the reluctance of people to disclose their disabilities. In the article we called for a reframing and a normalising of disability in the context of inclusion and diversity. Justin was a true fighter in so many ways and I hope his advocacy for disability rights will form part of how he is remembered."

Rachel then read an excerpt from Fighting Fate, which was painstakingly written by Justin using eye gaze technology.

Victoria Shephard: What is Motor Neurone Disease?

Currently over 2,000 Australians are living with motor neurone disease (MND), otherwise known as amyotropic lateral sclerosis (ALS). It is a neurodegenerative disease that affects specialised nerve cells called motor neurones, which control movement, including the ability to walk, talk, swallow and breathe. In MND the neurones die off and eventually cause paralysis. This is caused by the accumulation of toxic protein aggregates inside the motor neurones.

For most people, the cause is unknown, but for about 10% of people it is genetically inherited. The first gene to be linked to MND was SOD1, which is related to about 20% of familial cases. Victoria described the normal process for making proteins in cells, and that when there is a mistake in the production, there is a system for removing the dysfunctional proteins. In MND, there is an imbalance of proteins and the cell machinery is overwhelmed, resulting in a build up of insoluble aggregates. This not only kills the affected neurone but also those nearby, thereby spreading the disease through the body.

Victoria explained how her lab studies the SOD1, and that they have discovered a novel mutant. She also told us how a microscope can be used to find these mutants in a diagnostic tool. Early diagnosis is really important because it allows for treatment before the disease progresses too far.

Tom Walker: Investigating the cause of MND

Tom described the work that the team has done on a protein called TDP-43, which is associated with the majority of MND cases. In the lab, they can add fluorescent tags to TDP-43 and then track it in the cells using microscopes with high powered lasers. He also told us about another important cell machinery protein called heat-shock protein that normally helps to keep cells healthy. The team at University of Wollongong have done some exciting experiments using TDP-43 and heat-shock protein. They have been able to visualise a change in behaviour of TDP-43 when heat-shock protein is added.

Dr Isabella Lambert-Smith: Therapeutic targets for MND

The University of Wollongong team have studied a combination of three small molecules as potential drugs to prevent misfolding in SOD1 protein - one of the proteins associated with familial MND. Their experiments showed very promising results. They have also investigated how to target the SOD1 in faulty motor neurones. This is a critical problem to solve because targeting one type of protein is like trying to target a star in the night sky. They have studied the use of an antibody detector and attached it to another protein that eliminates the faulty SOD1. A therapy based on this approach could rescue motor neurones before they deteriorate.

Isabella explained that diagnosis of MND is very difficult, because for most people there is no genetic link. Often most of the motor neurones have been lost to the disease by the time a person begins receiving treatment. An effective treatment, as Justin envisioned, would therefore not only rescue dying motor neurones, but also replace those that have been lost. The team has been working on a novel concept to generate a new motor neurone starting with a sensory neurone. This is feasible because there are many more sensory than motor neurones in the body. The researchers are investigating how to "turn off" the genes that make neurone sensory and at the same time "turn on" genes for conversion into a motor neurone.

Fighting Fate

To learn more about Professor Yerbury's life and research, I encourage you to read his book! You can purchase it here:

Buy Fighting Fate and support MND research

Acknowledgements

The Yerbury Group at University of Wollongong: Professor Justin Yerbury AM, Dr Luke McAlary, Dr Jeremy Lum, Dr Isabella Lambert-Smith, Natalie Farrawell, Christen Chisholm, Victoria Shephard, Tom Walker, Dr Tracey Berg, Dr Rachael Bartlett-Johnson, Mikayla Brown, Siobhan Suters, Vanessa Hollingsworth.

Supporters and collaborators: Fight MND, MND Research Australia, Molecular Horizons, NHMRC, Flinders University, Ooi Group, Ecroyd Group, Wilson Group, Dr Yee Lian Chew.