One in 10 people diagnosed will have early-onset Parkinson’s disease, developing disease before the age of 50.
By the time most people with Parkinson’s disease are diagnosed, a large proportion of dopamine-producing nerve cells have already been lost.
Many of the symptoms of Parkinson’s disease develop when nerve cells that make dopamine are destroyed. Dopamine is a chemical messenger that relays messages and plays a key role in controlling movement.
If the brain does not have enough dopamine, movements can become slow and tremors can develop. It is still unclear what triggers Parkinson’s disease to develop, but researchers are investigating the role of inflammation, environmental triggers and genetic factors.
Symptoms can be very varied, and include tremors, slow movement, imbalance and stooped posture. Parkinson’s disease is also linked to sensory symptoms such as depression and problems with memory and thinking.
As many as 80 per cent of people with Parkinson’s disease will develop a type of dementia, related to the build-up of proteins in the brain.
The disease is more common in people over 60 years of age, although 10-20 per cent of people have early-onset Parkinson’s disease before the age of 50.
There are a number of medications that help people with Parkinson’s disease to manage their symptoms. These therapies can help to alleviate symptoms, but cannot slow or stop disease progression.
Unfortunately, by the time symptoms appear and patients are diagnosed, much of the nerve cell loss has already happened. There is currently no cure for Parkinson’s.
WEHI researchers Dr Jonathan Bernardini and Associate Professor Grant Dewson have discovered how a protein linked to Parkinson’s disease may protect nerve cells in the brain.
Parkin is a protein that is absent or faulty in many cases of early-onset Parkinson’s disease. In a healthy brain, Parkin helps keep cells alive, so they can repair damage to mitochondria, which supply energy to cells.
The researchers showed that Parkin works with cell death molecules, ‘buying time’ for the cell to repair damaged mitochondria before the cell is ‘sacrificed’.
If Parkin is faulty – such as in early-onset Parkinson’s disease – it can’t perform this role, and excessive cell death can occur. This unrestrained cell death may contribute to nerve cell loss in Parkinson’s disease.
”“In brain cells with normal Parkin, cell death is delayed. Parkin ‘buys time’, allowing the cell’s repair mechanisms to respond to the damage rather than just killing the cell.”
– Associate Professor Grant Dewson
Drugs that, for example, mimic or promote the effect of Parkin may be able to reduce harmful cell death in the brain, slowing or even halting dementia progression.
Using innovative technologies, we are studying how and why nerve cells die, and investigating critical proteins that keep nerve cells in the brain alive and healthy.
Our researchers are also using the latest mathematical and computational tools to understand the genetic basis of Parkinson’s disease.
This is particularly focused on two proteins – Parkin and ubiquitin – which, when abnormal or improperly controlled, drive Parkinson’s disease.
Understanding how these proteins function in healthy and diseased cells will enable us to identify whether they are good targets for developing treatments.
Our clinician-researchers bring together their medical and scientific expertise to lead clinical studies searching for early indicators of dementia in patients with Parkinson’s disease.
They are also using advanced technologies to study samples from healthy people who go on to develop Parkinson’s disease over time, to find ‘signatures’ that could be developed into a blood test for the disease.
Currently there is no cure for Parkinson’s disease, and available treatments only alleviate symptoms; they do not halt or slow disease progression.
Our collaborative approach – together with WEHI’s National Drug Discovery Centre – uses the latest technology and discovery approaches to test chemical compounds that may be developed as new treatments for Parkinson’s disease.
Our history includes transformative discoveries that underpin new medicines that have changed millions of lives.
WEHI researchers discover adult brains contain stem cells that can produce new neurons. Using a cocktail of hormones known as growth factors, they stimulate the growth of new brain cells, pointing towards the presence of stem cells inside the brain.
It overturned a long-held belief that we are born with all the neurons we will ever have, and although the neurons continue to make new connections throughout our lives, their number will slowly decline.
Triggering growth of new neurons could hold the key to repairing damage caused by neurodegenerative conditions.
WEHI researchers discover a new class of proteins, called inhibitors of apoptosis (IAP) proteins, that play a role in preventing cell death.
As well as leading to potential anti-cancer treatments, this research could pave the way for blocking cell death in neurodegenerative diseases.
A team of scientists at WEHI are striving to understand how the protein BCL-2 keeps cells alive. They discover a previously unknown protein, BIM, that triggers cell death. Although BIM can force cells to die, normal healthy cells can somehow stifle BIM’s deadly behaviour. Our researchers reveal that BIM can be sequestered in a harmless form in cells, and only released in certain lethal situations, triggering rapid cell death.
It provides new insights into the control of cell death, and its role in cancer, autoimmune diseases and neurodegeneration.
After more than 10 years of effort, WEHI researchers purify brain stem cells. They use a technique called flow cytometry to identify stem cells based on molecules on the cell surface – similar to scanning the barcode of groceries in a supermarket. While the technique is routine now, it was very advanced at the time.
The brain stem cell has the capacity to produce very large numbers of nerve cells. The discovery sparks great excitement that in the future brain stem cells may be harnessed as a treatment for traumatic brain damage and neurodegenerative conditions.
Our scientists use powerful X-ray beams at the Australian Synchrotron to visualise how a critical cell death protein, BAX, punches holes in the mitochondrial membrane, thus condemning the cell to die.
Defects in cell death have been linked to diseases such as cancer and neurodegenerative conditions. With this detailed blueprint, they can begin to design potential new treatments for degenerative diseases like Parkinson’s, by blocking the activity of BAX to prevent cell death.
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