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Axon Degeneration And Axon Protection In CNS Disease And Injury
Funder
National Health and Medical Research Council
Funding Amount
$389,120.00
Summary
One of the major reasons for the clinical symptoms of neurological diseases such as Alzheimer’s disease and Motor Neuron Disease is the loss of connections between the nerve cells. Nerve cells are connected by specialized processes called axons. In disease these processes can breakdown. This project specifically looks at how axons break down in disease and tests therapeutic strategies to protect them.
Trials of numerous agents to slow the progression of Parkinsons disease have provided ambiguous or negative results despite having good preliminary evidence for their efficacy. The most likely reason is that many nerve cells are already destroyed by the time of diagnosis. Thus effective therapies may be most (and possible only) effective when administered in the presymptomatic stages of disease. This proposal is directed at developing method to detect early presymptomatic Parkinsons disease.
Cognition In Motion: Characterization And Evolution Of Cognitive Dysfunction In Motor Neurodegeneration And Frontotemporal Dementia
Funder
National Health and Medical Research Council
Funding Amount
$604,106.00
Summary
Motor neuron disease (MND) is a fatal disease. Cognition may be unaffected or may be severely impaired to warrant a dementia diagnosis. The cognitive status at onset, its progression and the presence of co-morbid dementia of most MND patients is unknown. This research program will develop and validate a cognitive screener that diagnosis co-morbid dementia in the context of motor neurodegeneration in MND.
Developing Insight Into The Molecular Origins Of Familial And Sporadic Frontotemporal Dementia And Amyotrophic Lateral Sclerosis
Funder
National Health and Medical Research Council
Funding Amount
$6,377,279.00
Summary
There is strong evidence that frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) represent a spectrum of neurodegenerative disease with common origins. A combined study of FTD/ALS patient cohorts will provide greater power to identify these shared molecular origins. We aim to discover gene variants that cause, predispose, or modify onset and progression of inherited and sporadic FTD/ALS, and validate and study our discoveries in new cell and animal models of these disorders.
BRAIN-MEND: Biological Resource Analysis To Identify New Mechanisms And Phenotypes In Neurodegenerative Diseases
Funder
National Health and Medical Research Council
Funding Amount
$861,866.00
Summary
Current classification of neurodegenerative diseases (ND) based on clinical phenotypes does not take into account underlying disease heterogeneity, or overlapping disease mechanisms, thus hindering therapy development. Segregation and re-classification of ND phenotypes is urgently needed. BRAIN-MEND will reclassify existing phenotypic classifications using using pathway and network analyses within and across complex NDs.
Therapeutic Targeting Of Neuroinflammation To Slow The Progression Of Neurodegenerative Disease
Funder
National Health and Medical Research Council
Funding Amount
$463,652.00
Summary
My research has identified key components of our immune system, that can worsen disease in conditions such as Parkinson’s disease and motor neuron disease. I hope that exploring these components in animal models, and patients suffering from these diseases, my group can identify new therapeutic drug candidates that can be progressed in clinical trials. Ultimately, this may lead to new treatments to reduce disease burden in patients suffering from these neurodegenerative conditions.
A Central Role For ER-Golgi Trafficking In Motor Neuron Disease
Funder
National Health and Medical Research Council
Funding Amount
$434,652.00
Summary
Amyotrophic lateral sclerosis (ALS) patients currently face a bleak future. In the common global form of disease, the average length of survival after diagnosis is 31 months. Current therapies have at best a modest effect on the course of the disease with little or no benefit in terms of overall patient survival. This study will address the basic underlying biochemical mechanisms of disease in both sporadic and genetic forms of ALS. This studies will lead to opportunities to develop new therapie ....Amyotrophic lateral sclerosis (ALS) patients currently face a bleak future. In the common global form of disease, the average length of survival after diagnosis is 31 months. Current therapies have at best a modest effect on the course of the disease with little or no benefit in terms of overall patient survival. This study will address the basic underlying biochemical mechanisms of disease in both sporadic and genetic forms of ALS. This studies will lead to opportunities to develop new therapies in the future.Read moreRead less
Bioenergetic Deficit In Neurodegeneration: Studies In Motor Neuron Disease (MND)
Funder
National Health and Medical Research Council
Funding Amount
$300,967.00
Summary
Motor neurone disease (MND) is a fatal neurodegenerative disease. There is no known cause and no known cure. We believe that a defect in the way in which the muscle and nerve cells generate energy to sustain survival causes for there to be added metabolic stress on their already high energetic load, ultimately leading to cell death. This project aims to understand the vicious cycle of energy deficiency that leads to the catastrophic events that cause the death of muscle and nerve cells.
Disruption To Intracellular Trafficking As A Central Pathogenic Mechanism In Amyotrophic Lateral Sclerosis (ALS)
Funder
National Health and Medical Research Council
Funding Amount
$688,157.00
Summary
There are several different forms of ALS (MND), but the disease appears very similar in terms of symptoms and pathology. We have identified a common disease process shared by several different forms of ALS, which suggests that this is the underlying mechanism by which motor neurons die. This study will investigate whether we can develop new drug targets based on this mechanism in animal disease models. This may ultimately assist in the development of new treatments for ALS.
Determining The Contribution Of Peripheral Immune Complement Signalling In The Progression Of Motor Neuron Disease
Funder
National Health and Medical Research Council
Funding Amount
$1,000,871.00
Summary
Motor neuron disease (MND) is a debilitating and lethal neurodegenerative disease, which has no effective treatment. We propose that an overactive immune system is a major contributing factor in this disease. To test this, we will examine immune pathways in patients suffering from MND, and will test a novel immune-modulatory drug in animal models of MND. We hope to identify new therapeutic avenues to slow disease progression, and improve the quality of life in patients diagnosed with MND.