In 1971, Perth hosted a major international congress on muscle diseases where the idea of establishing a neuromuscular research institute in Perth was born. This culminated in the establishment in 1982 of the Australian Neuromuscular Research Institute with Professor Kakulas as its Foundation Director.
1960
The Quokka Story
Muscular Dystrophy Western Australia (MDWA) and the Perron Institute, originally the Australian Neuromuscular Research Institute (ANRI) – owe their origins to the humble Rottnest Island quokka.
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The quokka, a marsupial species about the size of a domestic cat that is found in small pockets on islands off the coast of Western Australia, in particular on Rottnest Island off Perth, has played a curious role in the history of muscular dystrophy research.
In the post-World War II period, the quokka was intensively investigated by scientists from the University of Western Australia because of its unique habitat and biology. However, this important research was hampered by the animals dying of muscle paralysis when kept in small cages on the university campus.
In 1960, Professor Byron Kakulas, then embarking on what was to become a stellar career in neuropathology, including the study of muscle disease, was asked to investigate the problem. Despite many theories to the contrary, Professor Kakulas began to believe that the muscle breakdown in the caged quokkas was due to anti-oxidant deficiency. He showed that it was possible to successfully treat paralysed quokkas with Vitamin E, in the process making the momentous discovery that complete muscle regeneration is achievable, something that was previously believed to be impossible because the prevailing theory was that muscle as a specialised tissue was incapable of regeneration after injury. The treated quokkas completely recovered and serial biopsies of their muscle revealed a sequence of changes leading to complete regeneration.
This world-shattering discovery completely reversed the prevailing dogma towards muscle diseases and especially to the muscular dystrophies, which had been previously considered to be hopelessly incurable.
Such was the importance of this discovery that a large international meeting was held in Perth with the object of promulgating the discovery and stimulating research worldwide, which was considerably accelerated as a result. This meeting, which took place in 1971, was the first major international congress to be held in Australia and put Australia’s medical research on the international map.
Built around the discovery of complete muscle regeneration in the quokka was the academic publication Principles of Myopathology as Illustrated in the Nutritional Myopathy of the Rottnest Quokka, which was adopted as the standard teaching of the subject worldwide. Much of the tremendous progress that has taken place in muscle research since 1960 can be traced back to the early work on the quokka.
The Muscular Dystrophy Association of Western Australia (MDAWA) was established in 1967 to raise money to find a cure based on the quokka discovery. Fund raising was spearheaded by the Rotary Club of West Perth and supported by the Chanel 7 Telethon Charitable trust. Later it was possible to widen the scope of research to include other neuromuscular diseases through the establishment of the Australian Neuromuscular Research Institute (ANRI), in 1983, now the Western Australian Research Institute (the Perron Institute).
The dream that muscular dystrophy could be cured was fulfilled by Professors Steve Wilton and Sue Fletcher of the institute who have applied elegant molecular techniques to override the mutation and correct the genetic defect underlying Duchenne muscular dystrophy. Their work is now undergoing clinical trials in the United States.
It can be said that there are very few instances in the history of medical research that have progressed from blindness to cure in one lifetime, in the case of muscular dystrophy in one of the most difficult fields of human disease.
The quokka research was the subject of Professor Kakulas’ doctoral thesis in 1963 and was subsequently published by the UWA Press (1982). A concise history of muscle research may be found in the journal, Neurology India (56: 258-262, 2008).
1967
Professor Byron Kakulas AO
In 1967, Professor Byron Kakulas, then Professor of Neuropathology at the University of Western Australia, in partnership with a group of parents whose children suffered from muscular dystrophy took the bold decision to establish the Muscular Dystrophy Association of Western Australia, now Muscular Dystrophy Western Australia.
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In 1967, Professor Byron Kakulas, then Professor of Neuropathology at the University of Western Australia, in partnership with a group of parents whose children suffered from muscular dystrophy took the bold decision to establish the Muscular Dystrophy Association of Western Australia, now Muscular Dystrophy Western Australia. The group’s goal was to raise funds for medical research on muscular dystrophy and were assisted in this process through the generous support of the West Perth Rotary Club and the Channel 7 Telethon Charitable Trust.
1978 – 1983
Foundation of a WA Neuromuscular Institute
In 1978, the Western Australian Government indicated that it was prepared to make space available in the old Sir Charles Gairdner Hospital building at the Queen Elizabeth II Medical Centre in Nedlands.
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With the establishment of the Australian Neuromuscular Research Institute in 1982, detailed planning began on the conversion of this space into medical research facilities, which were completed over the period 1982–1990. The institute’s first research work began in part of the old building in 1983.
1983 to 2017
From ANRI to Perron Institute
The institute, renamed the Western Australian Neuroscience Research Institute in 2012, and then in 2017 proudly became the Perron Institute for Neurological and Translational Science in honour of Mr Stan Perron AC and his family’s long-standing support.
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Since then, the Perron Institute has continued to grow and now involves research on over fifteen neuromuscular and neurological disorders. The goal of the research continues to be translational, so that findings from the laboratory lead to better clinical outcomes for people suffering.
Muscular Dystrophy Western Australia has continued to contribute towards to the institute’s funding for many years and even now continues a close association with the research of Professor Wilton and Fletcher on muscular dystrophy. In 2017 Muscular Dystrophy WA celebrated its 50th Anniversary.
An Interview with Byron Kakulas AO
Emeritus Professor Byron Kakulas AO, reflects on his career
Perron Institute Founding Director Emeritus Professor Byron Kakulas AO, neuropathologist, reflects on his career and momentous discoveries that led to a treatment for sufferers of Duchenne muscular dystrophy.
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