Models Of Cerebellar Function During Motor Learning
Funder
National Health and Medical Research Council
Funding Amount
$384,775.00
Summary
The cerebellum is responsible for smooth movements and for learning new patterns of motions, but many details of how it works are unclear. We aim to describe neural activity in the cerebellum while motor learning is in progress, and determine exactly what patterns of activity lead to the acquisition of new types of learned movement. The basic knowledge gained will contribute to a better understanding of cerebellar disorders including nystagmus, ataxia, and loss of motor control with ageing.
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.
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
Understanding The Role Of TDP-43 In Motor Neuron Disease.
Funder
National Health and Medical Research Council
Funding Amount
$654,091.00
Summary
Motor neuron disease (MND) is a fatal neurodegenerative disease with no cure. The cause of MND is poorly understood but new research has shown that defects in TDP-43, an RNA binding protein involved in gene regulation, can lead to the disease. This project is aimed at discovering the molecular mechanisms of TDP-43 function, which will improve the understanding of the disease and aid in the development of new therapies.
The Role Of Central And Peripheral Synaptic Activity In The Developmental Death Of Motoneurons.
Funder
National Health and Medical Research Council
Funding Amount
$463,145.00
Summary
Information processing in the nervous system relies on the effective communication between neurons and their target cells which make up our neuronal circuitry. At the centre of all this is the synapse, the specialized contact between a neuron and its target cell, be it another neuron in the brain or a target organ such as skeletal muscle. Our primary goal is to determine how the formation of synaptic connections during development regulates neuronal survival. In this proposal we have focussed on ....Information processing in the nervous system relies on the effective communication between neurons and their target cells which make up our neuronal circuitry. At the centre of all this is the synapse, the specialized contact between a neuron and its target cell, be it another neuron in the brain or a target organ such as skeletal muscle. Our primary goal is to determine how the formation of synaptic connections during development regulates neuronal survival. In this proposal we have focussed on the neuromotor system as it is a well characterised part of the nervous system. During development, 50% of motoneurons die at a time when they are making contact with skeletal muscle, and when contacts onto motoneurons by other neurons are being established. We believe that the formation of effective synaptic contacts onto motoneurons, as well as connections by motoneurons onto muscle are the key regulators of motoneuron survival. We are in a position to be able to address what regulates motoneuron death; as we have a number of mice which lack key molecules needed for the formation of specialisations that allow neuronal contacts to be made between motor neurons and their muscle, and with other neurons within the spinal cord. By examining the function of motoneurons, counting them and screening for molecular changes in these mice, we will be able to dissect out the mechanism of how a motoneurons' fate is determined during developmental motoneuron death. This research could help in developing strategies aimed at improving neuronal connections to improve neuronal viability. Our research will have important implications for our understanding about the basis of adult neuro-degenerative diseases, such as motor neuron disease and Alzheimer's, which are in part characterised by a molecular breakdown in neuronal connections that ultimately result in neuronal death.Read moreRead less