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.
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.
Validating The NLRP3 Inflammasome As A Therapeutic Target In Motor Neuron Disease
Funder
National Health and Medical Research Council
Funding Amount
$780,249.00
Summary
There is no effective treatment for Motor Neuron Disease (MND). Two Australian’s die each day from MND, and 2 more are diagnosed. Modification of the immune system is one potential means of slowing MND. Our research team has identified a component of the immune system, which may be an ideal therapeutic target to treat this disease. Our project will test this therapeutic target in animal models of MND, and in human MND blood samples.
Targeting Autophagy To Improve Protein Metabolism In ALS
Funder
National Health and Medical Research Council
Funding Amount
$586,001.00
Summary
One common feature of MND is the accumulation of protein deposits inside nerve cells which leads to their death. We have identified a potent drug which enhances autophagy, a protective process which breaks down protein deposits inside cells. We have shown that this autophagy enhancer efficiently clears protein deposits linked to MND in the Petri dish. We propose to treat MND mice with this autophagy enhancer and predict that it will slow disease signs, preserve lifespan and protect nerve cells.
Investigating The Pathogenesis Of Motor Neuron Disease Using Zebrafish Models
Funder
National Health and Medical Research Council
Funding Amount
$554,881.00
Summary
Amyotrophic lateral sclerosis (ALS) causes the progressive death of motor nerves. Most ALS patients die within 2-5 years of disease onset. We recently identified mutations that cause ALS in the genes TDP-43 and FUS. We aim to develop fish that carry these mutations. We will monitor and test this fish to establish whether they develop similar symptoms and features to ALS. If so, this new fish model will be available for investigating the biology of the disease and for evaluating treatments
The Role Of Excitotoxicity In Mediating Distal Axonal Degneration In ALS
Funder
National Health and Medical Research Council
Funding Amount
$392,952.00
Summary
Amyotrophic lateral sclerosis (ALS), the major cause of motor neuron disease, is a devastating diseasse for which there is no cure. There have been significant advances in understanding the pathology of ALS yet we still don’t know what causes the dying back of spinal motor neurons. We have new evidence that suggests that ALS may, in part, be caused by excitotoxcity - or over stimulation - of neurons in the spinal cord. We will follow this lead using a range of cutting edge experimental models.
Pathophysiology, Site Of Disease Onset And Mechanisms Of Spread In Amyotrophic Lateral Sclerosis
Funder
National Health and Medical Research Council
Funding Amount
$704,270.00
Summary
Amyotrophic lateral sclerosis (ALS) is a fatal disease of the human motor system resulting in progressive paralysis of voluntary muscles and death within 5 years of symptom onset. A key unresolved issue in the understanding of where ALS starts and what factors drive the disease. This project will utilise sophisticated techniques to determine the site of onset, factors controlling disease spread and ultimately identify treatment targets that could slow down or even stop disease progression.