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  • Funded Activity

    Neurodegenerative Diseases Of The Ageing Brain: Diagnosis & Therapy Based On The Study Of Aggregated Protein Deposition

    Funder
    National Health and Medical Research Council
    Funding Amount
    $5,248,710.00
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    Funded Activity

    Characterization Ol A Novel Covalently Cross -linked Abeta Peptide Dimer And Its Role In Alzheimers Disease.

    Funder
    National Health and Medical Research Council
    Funding Amount
    $553,236.00
    Summary
    Currently there are limited therapeutic treatments and no cure for Alzheimer's disease (AD). The key protein causing AD is called Abeta. Abeta peptides form dityrosine cross-linked dimers (when 2 peptides join together) and this is thought to be responsible for killing brain cells in AD. Therefore, this proposal will determine the role of Abeta dimers in relation to killing brain cells and the progression of AD through analysis of their biological and biochemical properties.
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    Funded Activity

    The Effect Of Metals On Neurofibrillary Tangle Formation

    Funder
    National Health and Medical Research Council
    Funding Amount
    $333,313.00
    Summary
    The majority of studies into Alzheimer's disease (AD) have focussed on two brain lesions- the plaque and neurofibrillary tangle (NFT), which are believed to have a causative role in AD. Our lab has made several seminal discoveries about the role that metals play in the development of plaques. We are now extending this work to evaluate the role of metals in NFT formation. These studies will provide insight into the formation and possible treatments for this primary brain lesion in AD.
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    Funded Activity

    An Analysis Of A Model Of Movement Disorder Lacking D1R Positive Neurons.

    Funder
    National Health and Medical Research Council
    Funding Amount
    $346,446.00
    Summary
    The experiments outlined in this project proposal are aimed at further characterizing a genetically engineered mouse the generation of which was originally funded by the Australian NH and MRC. The mutant mouse suffers from the loss of brain cells in a part of the brain called the striatum. The mouse model will allow us to understand how damage to brain structures cause disabling human neurodegenerative diseases such as Parkinsonism and Huntington's disease. The mouse model is unique as the mice .... The experiments outlined in this project proposal are aimed at further characterizing a genetically engineered mouse the generation of which was originally funded by the Australian NH and MRC. The mutant mouse suffers from the loss of brain cells in a part of the brain called the striatum. The mouse model will allow us to understand how damage to brain structures cause disabling human neurodegenerative diseases such as Parkinsonism and Huntington's disease. The mouse model is unique as the mice suffer from the same type of movement abnormalities which afflict individuals with this spectrum of neurological illnesses. We will look at both structural changes in the brain as well as brain function as defined by the behavioural responses of the damaged brain to drug administration. The experiments also focus on the ultimate correction of the neurological deficits by transplantation of purified nerve cell progenitor cells.
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    Funded Activity

    GENETIC FACTORS AND REGIONAL BRAIN ATROPHY IN THE DIAGNOSIS OF DEMENTIA WITH LEWY BODIES

    Funder
    National Health and Medical Research Council
    Funding Amount
    $605,151.00
    Summary
    The number of people with dementia is increasing in Australia as people live longer. Dementia sometimes has a genetic basis and identification of such cases has improved our understanding of the events leading to the destruction of the brain tissue. In the vast majority of people, the degenerative changes were previously thought to be as a result of Alzheimer's disease. However, our recent research, funded by the NHMRC, confirms international findings showing more than 25% of people with dementi .... The number of people with dementia is increasing in Australia as people live longer. Dementia sometimes has a genetic basis and identification of such cases has improved our understanding of the events leading to the destruction of the brain tissue. In the vast majority of people, the degenerative changes were previously thought to be as a result of Alzheimer's disease. However, our recent research, funded by the NHMRC, confirms international findings showing more than 25% of people with dementia have a different disease called Dementia with Lewy bodies or DLB. Of course identifying these patients occurs at death when the cells in the brain can be examined for Lewy bodies. We now know that the brain degeneration differs significantly in patients with this disease. However, it is still not possible to identify DLB in life with any certainty. This project aims to develop objective methods to clinically differentiate dementia patients. We will seek out families in which genetic influences may underly the disease and determine whether these factors differ from those found in other dementing illnesses. Also, our preliminary studies have observed volume loss in a particular brain region in pathologically confirmed DLB patients. We wish to do further measurements to determine if tissue loss in this region can clinically differentiate DLB patients. In addition, we will determine the reasons for the tissue loss by careful pathological studies.
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    Deciphering The Neuroprotective Mechanism Of Parkinsons Disease-Associated Protein Kinase PINK1

    Funder
    National Health and Medical Research Council
    Funding Amount
    $547,994.00
    Summary
    Parkinson's disease is caused by premature death of nerve cells that control body movements. The enzyme PINK1 protects against nerve cell death by chemically modifying specific cellular proteins that maintain cell survival. We aim at identifying these proteins and investigating how PINK1-catalysed modification modulates their ability to maintain nerve cell survival. The study will benefit development of drugs that protect against nerve cell death for treatment and prevention of the disease.
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    Funded Activity

    Targeting Inflammatory Mechanisms In Alzheimer's Disease.

    Funder
    National Health and Medical Research Council
    Funding Amount
    $392,750.00
    Summary
    Alzheimer s disease accounts for the majority of dementia cases and is the most common cause for nursing home requirements in Australia. It is not a disease that is confined to old age as it can also affect individuals in their 20s and 30s. There is currently no cure for Alzheimer's disease, largely because the underlying cause is unknown. Deposition of the amyloid-beta protein within the brain of Alzheimer's disease patients is thought to be responsible for the neuronal cell loss which underlie .... Alzheimer s disease accounts for the majority of dementia cases and is the most common cause for nursing home requirements in Australia. It is not a disease that is confined to old age as it can also affect individuals in their 20s and 30s. There is currently no cure for Alzheimer's disease, largely because the underlying cause is unknown. Deposition of the amyloid-beta protein within the brain of Alzheimer's disease patients is thought to be responsible for the neuronal cell loss which underlies the dementia. However, amyloid-beta protein deposition can occur in the absence of dementia and in the asbence of significant neuronal cell loss, suggesting that an alternative mechanism of neurotoxicity exists. Inflammation is a consistent feature of the Alzheimer's disease brain. We have preliminary evidence to suggest that inflammation is responsible for the neurotoxicity in Alzheimer's disease. We have recently observed a significant inflammatory response surrounding an unidentified protein in the brains of individuals with a familial form of Alzheimer's disease due to a genetic mutation. This inflammatory response is not associated with the significant amyloid-beta protein deposition seen in these cases suggesting that a novel potent inflammatory stimulus exists. Furthermore, these cases have greater neuronal cell loss and a shorter disease duration, both indicators of increased neurotoxicity. The present study is designed to determine the toxicity of inflammation and the stimulus driving this response in the Alzheimer's disease brain using tools for protein and gene analysis, as well as determining the extent of inflammation-mediated toxicity on neuronal cells grown in culture. Only by addressing these aims can we concentrate on developing safe and effective therapeutic strategies to prevent or treat the disease process.
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    Funded Activity

    The Influence Of Gamma-secretase Complex Subunits On The Production Of Alzheimer Amyloid Peptides

    Funder
    National Health and Medical Research Council
    Funding Amount
    $641,540.00
    Summary
    Alzheimer's disease is a devastating illness of the aged population and represents a major socio-economic problem in Australia. There is no cure or effective treatment for the illness. A toxic protein fragment known as Abeta amyloid accumulates in the brain of the diseased patients. This is produced by an enzyme called gamma-secretase. This project will investigate different forms of gamma-secretase to gain information useful for developing Alzheimer-specific drugs with little side-effects.
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    Funded Activity

    Ghrelins Novel Neuroprotective Effects In Parkinsons Disease Are Mediated By AMP-activated Protein Kinase (AMPK).

    Funder
    National Health and Medical Research Council
    Funding Amount
    $400,885.00
    Summary
    Studies show that body mass index, midlife adiposity and diabetes are associated with Parkinson's disease (PD). During obesity there is a dramatic change in nutritional information, such as hormones, sugars and fats, carried in the blood. This proposal explores how this altered nutritional information in obesity kills the brain cells associated with PD. It will examine how ghrelin, a metabolic hormone inversely related to obesity, influences and protects brain cell activity in models of PD.
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    Funded Activity

    Motor Neurone Disease - Pathophysiological Insights Into The Site Of Origin And Patterns Of Neurodegeneration.

    Funder
    National Health and Medical Research Council
    Funding Amount
    $309,361.00
    Summary
    Motor neurone disease (MND) kills one Australian per day and is characterised by progressive loss of the corticospinal tract, that controls all voluntary movements. The present project will investigate where MND begins and document how the loss of nerves within the brain, spinal cord and peripheral nerves evolves. In addition to providing information about prognosis, new quantifiable measures will be developed to objectively monitor patients in future treatment and prevention trials.
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