Investigating The Involvement Of Human Derived Astrocytes And Motor Neurons In The Pathology Of Motor Neuron Disease.
Funder
National Health and Medical Research Council
Funding Amount
$287,321.00
Summary
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease, which results in the death of nerves that innervate muscle, known as motor neurons. Recent studies using mouse ALS models showed that certain cells that normally support motor neurons may be directly contributing to their death in ALS. We propose to derive ALS-diseased human cells and investigate how these cells may react in ‘normal’ tissue. These studies are clinically relevant in understanding ALS pathological processes.
Hereditary Motor Neuronopathies And Neuropathies: Mechanisms Of Neurodegeneration And Potential Modification.
Funder
National Health and Medical Research Council
Funding Amount
$104,664.00
Summary
The aim of the study is to investigate the pathophysiology of motor neuron degeneration in its various acquired and inherited forms. Nerve function will be determined by clinical and neurophysiological assessments in patients. The findings of the study will reveal further insights into the cause and progression of disorders of the motor neuron. This data may function to monitor disease progression, response to potential therapies and lead to the development of further therapeutic strategies.
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.