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Mitochondria: our bodies' Jekyll and Hyde |
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Mitochondria, say La Trobe University researchers, Dr Mike Ryan and Dr Ann Frazier, are the Jekyll and Hyde of our bodies.
Mitochondria are organelles, highly specialised structures essential to the viability of our cells - but, like the fictional scientist and his evil alter-ego, the activity of mitochondria can be both good and bad.
These little powerhouses play a fundamental role in providing energy for the cell but they also contain proteins that can be 'poisonous' and, when released, cause the death of the cell.
Based at La Trobe's School of Molecular Sciences, Mike and Ann have embarked on a four-year research project aimed at improving understanding of the machinery by which mitochondria operate.
There are many important long term implications in this work, including the treatment and possible prevention of mitochondrial disorders as well as Parkinson's disease and some forms of cancer.
Mike, a senior lecturer who specialises in mitochondrial biogenesis, explained that mitochondria are like energisers in our cells. "They are complex because they not only grow and divide, but they also fuse together. These morphology changes are important for supplying energy to all parts of the cell," Mike said.
"We are looking at the machinery involved in how they divide and how they distribute themselves inside our cells. If we know the basics, we can understand how changes in mitochondrial morphology occur in more specialised cells.
"For example, sperm cells contain fused mitochondria that wrap around the base of the tail and supply energy for their swimming," explained Mike.
But what about the Jekyll and Hyde factor?
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Dr Ann Frazier and
Dr Mike Ryan
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The body benefits from the energy provided by mitochondria, but it also suffers when they play their other role - causing cell death. Regulated cell death is important during embryonic development, and in removing old cells in our bodies as new ones are made. The problem is that cancers also form when not enough cell death takes place. There is evidence that cell death is caused by changes in the morphology of mitochondria.
Mike's team is collaborating with researchers from Melbourne's Royal Children's Hospital (RCH), with the aim of helping babies born with a mitochondrial disease. One out of 5 000 babies born worldwide suffers from a mitochondrial disease.
Mitochondrial disease affects about 1 000 people in Australia. Each year, around 50 children develop the condition and more than half die before adulthood; babies with the most common form of the disease die within the first few weeks of their lives.
Such diseases are classified as energy generation disorders and result in babies being unable to sufficiently utilise their muscles and nerves. There are also common neurodegenerative disorders associated with abnormal mitochondrial metabolism, often resulting in a fatal outcome.
The La Trobe team is working with Dr David Thorburn of RCH's Murdoch Children's Research Institute, examining skin cells from patients with mitochondrial disease to determine which defects are present. Both aspects of mitochondrial biogenesis are funded by separate grants from the National Health & Medical Research Council.
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