The Pathogenesis Of Motor Neuron Degeneration Caused By A Triplet Repeat Expansion In The Androgen Receptor Gene.
Funder
National Health and Medical Research Council
Funding Amount
$284,748.00
Summary
Male sex hormones, or androgens, work by binding to a specific receptor, known as the androgen receptor. Androgens have an important and yet poorly understood role in nerve function. Our research is investigating how a genetic mutation in the androgen receptor causes Kennedy?s disease. This is a rare disease, affecting adult males, which causes nerves to die. The nerves which are affected are those supplying our muscles, called motor neurons. This leads to muscle wasting in the face and body. Ot ....Male sex hormones, or androgens, work by binding to a specific receptor, known as the androgen receptor. Androgens have an important and yet poorly understood role in nerve function. Our research is investigating how a genetic mutation in the androgen receptor causes Kennedy?s disease. This is a rare disease, affecting adult males, which causes nerves to die. The nerves which are affected are those supplying our muscles, called motor neurons. This leads to muscle wasting in the face and body. Other symptoms include testicular wasting, reduced fertility and breast tissue enlargement. It is currently not known what causes motor nerves to degenerate in Kennedy?s disease. We are endeavouring to investigate the cause of Kennedy?s disease via the generation of a transgenic mouse carrying this mutation. It is only through a studying transgenic mouse affected by this disease can we begin to understand what is happening to nerves to cause them to die, and importantly, how can we prevent them from dying. These studies will also provide crucial information on the effects of sex hormones on nerves. As there is currently no treatment for Kennedy?s disease, an aim of this project is to investigate how we can treat this disease. This will be the first time that we can systemically test potential treatments and work toward preventing the degeneration of these nerves. Kennedy?s disease is related to a number of other neurodegenerative diseases including Huntington?s disease, which are caused by similar genetic mutations. All of these diseases are caused by degeneration of specific nerve cells. Evidence suggests that there may be similar mechanisms involved in all of these diseases. The results of this study will therefore help us to understand a range of diseases and may eventually lead to the development of therapeutic strategies to prevent their debilitating effects.Read moreRead less
Molecular Mechanisms Mediating Experience-dependent Cellular Plasticity And Cognitive Deficits In Huntingtons Disease
Funder
National Health and Medical Research Council
Funding Amount
$550,387.00
Summary
We will use a genetic mouse model of Huntington's disease (HD), to understand how cognitive disorders (dementia) are caused, focusing on cells and molecules within the brain. We will investigate how the HD gene mutation disrupts communication between brain cells (neurons), as well as disrupting production of new cells (via adult neural stem cells). The results of this project will not only have implications for treating HD but also for other diseases involving dementia, such as Alzheimer's.
Maintenance And Operation Of The Australian Seafood Extension And Advisory Service (AUSEAS)
Funder
Fisheries Research and Development Corporation
Funding Amount
$310,872.22
Summary
Objectives: 1. To provide the post-harvest sector of the Australian seafood industry with technical information and advice that is timely, accurate and relevant. 2. To proactively promulgate the results of research to companies that are well placed to use them. 3. To identify areas where research is needed and the institutes where the work can be done. 4. To promote networking between companies in the post-harvest area by personal contact and publications. 5. To provi ....Objectives: 1. To provide the post-harvest sector of the Australian seafood industry with technical information and advice that is timely, accurate and relevant. 2. To proactively promulgate the results of research to companies that are well placed to use them. 3. To identify areas where research is needed and the institutes where the work can be done. 4. To promote networking between companies in the post-harvest area by personal contact and publications. 5. To provide sales support for selected publications and database maintenance. These include directories, research reports and the FishMAD service, disseminating the results of the national seafood consumption study. 6. Publication of second edition of Australian Seafood Industry Directory 1997-99. Read moreRead less
Investigating Drug Treatments For A Machado Joseph Disease Using Transgenic Zebrafish
Funder
National Health and Medical Research Council
Funding Amount
$443,425.00
Summary
Machado Joseph disease (MJD) is a hereditary neurodegenerative disease that causes problems with a patient’s co-ordination and movement, leading to paralysis and death. Although the disease affects patients throughout the world, it is most common within Aboriginal communities of Arnhem Land in the Northern Territory. This project seeks to identify a drug treatment for the disease by examining the effect of relevant drugs on zebrafish genetically modified to have the human gene that causes MJD.
Calpeptin, And Related Candidates, For The Treatment Of Machado Joseph Disease
Funder
National Health and Medical Research Council
Funding Amount
$888,040.00
Summary
Machado Joseph Disease (MJD) is a neurodegenerative disease that causes impaired movement and progressive paralysis, leading to patient death. MJD is inherited within families, including a high number of Indigenous families of northeast Arnhem Land. We have identified a possible treatment for MJD that has positive effects on a small animal model of the disease (zebrafish carrying the human MJD gene). We plan to test this treatment further with the aim of developing a treatment for MJD patients.
Defining FMR1 And SNRPN Epigenetic Signatures Associated With Neurodevelopmental Disorders
Funder
National Health and Medical Research Council
Funding Amount
$318,768.00
Summary
Fragile X Syndrome and imprinting disorders such as Prader-Willi Syndrome and Angelman Syndrome are characterised by variable penetrance for intellectual disability, motor delay and autism spectrum disorder. This project aims to investigate the prognostic value of using blood-based biomarker tests and sensitive neuroscience informed measures to predict risk and severity of neuropsychological problems in children affected by these disorders.