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Protein Deposits: the black Holes of motor neurons

Dr Justin Yerbury and Isabella Lambert-SmithPrincipal Research Fellow Dr Justin Yerbury and PhD candidate Isabella Lambert-Smith. Photo by Simon Bullard.

A five-year international research project led by IHMRI scientists is making progress into the causes of motor neurone disease (MND).

According to the MND Association, about 2000 Australians, including almost 30 in the Illawarra-Shoalhaven, suffer from the disorder in which the nerve cells (neurones) controlling the muscles that enable us to move, speak, breathe and swallow begin to degenerate and die.

There is no effective treatment or cure and most people with MND die within three to five years of diagnosis. In rare cases, like that of eminent physicist and cosmologist Professor Stephen Hawking, life span is much longer.

The research, which is led by Principal Research Fellow Dr Justin Yerbury with the assistance of Senior Professor Mark Wilson and PhD student Isabella Lambert-Smith, from the University of Wollongong’s School of Biological Sciences, focuses on the protein molecules found in motor neurons.

Proteins are important because they are the second most abundant molecule in the body after water. The researchers discovered proteins that do not normally interact will gather into deposits within motor neurons.

Dr Yerbury, who met with Professor Stephen Hawking at the University of Cambridge in April, said a strong analogy can be made between the research findings and Professor Hawking’s work in physics and cosmology.

“There are almost as many protein molecules in a human body as there are stars in the universe and we liken the deposits of proteins within motor neurons to black holes,” he said. “They are dense accumulations that attract a whole range of proteins that become lost to the cell when they are sucked in. The biological properties of motor neurons make them vulnerable to this attack."

“In this study we wanted to chip away at one of the biggest questions about this disease—why do motor neurons die while other groups of neurons and tissues in the body remain unaffected. We believe that motor neurons are particularly vulnerable to losing proteins to their cellular black holes. This has brought us one step closer to explaining why only motor neurons die off in MND.”

Dr Yerbury, Senior Professor Wilson and Ms Lambert-Smith, who worked with researchers from the University of Cambridge; CRG Barcelona; Universitat Pompeu Fabra; University of NSW; and North Western University, hope this new understanding of the molecular origins of MND will provide a target for more effective treatment or therapies in the future.

They are now actively testing potential therapeutics with Research Fellow Dr Kara Vine from IHMRI's Drug Discovery Group.

This research has received international attention, with publication in top-ranking international journal, PNAS (Proceedings of the National Academy of Sciences of the United States of America), April 10, 2017, doi:10.1073/pnas.1613854114. The article title is “Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS”. Read the full article.

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NINE News Wollongong

Last reviewed: 9 June, 2017

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