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.
Unravelling The Molecular Basis Of Amyotrophic Lateral Sclerosis
Funder
National Health and Medical Research Council
Funding Amount
$342,325.00
Summary
The only known causes of ALS are gene mutations. State-of-the-art technologies will be used to find genetic causes of ALS to add to existing diagnostic testing and facilitate investigation into disease mechanism. ALS patients experience different disease courses, with variable age of onset, progression and duration of disease even among those with identical gene mutations. We will examine a well-characterised ALS patient cohort with differing disease manifestations to identify disease modifiers.
Language Deficits And Cognitive Dysfunction In Motor Neuron Disease
Funder
National Health and Medical Research Council
Funding Amount
$263,513.00
Summary
Motor neuron disease (MND) and frontotemporal dementia (FTD) are fatal degenerative brain diseases that overlap clinically and pathologically. Patients with either disease can develop a combination of motor and cognitive deficits (known as FTD-MND). Recent studies suggest that FTD-MND is more common in familial MND. This project will investigate language disturbance – a prominent feature of FTD – in MND patients to establish whether such impairment predicts the development of FTD-MND.
Neural Signatures Of Disease Spread And Evolution In Motor Neurodegenerative Syndromes
Funder
National Health and Medical Research Council
Funding Amount
$408,768.00
Summary
A lack of understanding of the earliest changes brought on by motor neurone disease (MND), otherwise known as amyotrophic lateral sclerosis (ALS), prevents early diagnosis and therapeutic intervention. The proposed project aims to comprehensively characterise neurological changes prior to disease onset in pre-symptomatic carriers with a known genetic mutation linked to MND using targeted neuropsychological assessments and advanced multi-modal neuroimaging to track disease progression.
Investigating Mechanisms Of Dementia And Motor Neuron Disease
Funder
National Health and Medical Research Council
Funding Amount
$303,924.00
Summary
Dementia and motor neuron disease (MND) are both neurodegenerative diseases which have devastating impacts on patients and their families. My research investigates the biochemical mechanisms involved in these diseases. Pathology in the nervous system of dementia and MND patients contains a protein called ‘TDP-43’, but it remains unknown how this causes disease. This project will therefore explore the effects of TDP-43 malfunction, which will provide insight into potential therapeutic strategies.