Sydney University Neuroscience

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Neurodegenerative Diseases

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Neurodegenerative Diseases

Associate Professor P Armati
Dr W Brooks
Professor W Burke
Dr J Kril
Associate Professor K North
Associate Professor N O'Dwyer
Dr R Pamphlett
Professor J Pollard
Professor J Prineas
Associate Professor R Taylor
Dr M Thomson

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This group studies both normal nerve structure and the effects on it of various diseases. Its research extends from individual nerve cells to muscle tissue and cognitive and behavioural changes.

Current studies examine the neuropathology of Alzheimer’s disease, virus transport within nerve cells, the effects of stroke and palsy on muscle, and neural changes in multiple sclerosis, alcoholism and muscular dystrophy.

Research Themes:

P Armati
Neurotoxicity and intra-neuronal virus transport. Myelin.

W Brooks • Link to Laboratory
Genetics of Alzheimer's disease and other dementias

W Burke
Myoclonus. Demyelination and remyelination. Effects of CNS lesions.

J Kril • Link to Laboratory
Neuropathology of the ageing brain: healthy ageing, vascular and
neurodegenerative disease.

K North
Cognitive and behavioural changes associated with neurofibromatosis; nemaline myopathy.

N O'Dwyer
Effects of stroke and palsy on muscle growth and contracture.

R Pamphlett


Motor neuron disease (known as ALS in the USA) kills one Australian every day. The disease causes a progressive loss of muscle strength, leading to death usually within 2 to 5 years. The weakness is due to a loss of motor neurons, the nerve cells in the spinal cord that activate muscle.


Motor neurons in the normal spinal cord

 


Loss of motor neurons in MND

 


In 10% of cases MND runs in families, but in 90% only one family member gets the disease, i.e. it is "sporadic". The cause of sporadic MND is not known, but we think that either a toxin or a virus enters motor nerve cells and destroys them over time.

Metal toxins are suspected in MND, since at low doses these toxins enter motor neurons selectively (i.e. other neurons are not affected). Possible environmental toxins in MND are lead, mercury, bismuth, cadmium or copper. We have found that about 40% of adult humans have metal toxins in their motor neurons. However, many people are exposed to toxins, yet few get MND. So if metals are a cause of MND, a genetic susceptibility to these toxins is likely to underlie the disease.

Viruses, especially those belonging to the family which includes poliovirus (the enteroviruses) have long been suspected in MND since they enter motor neurons selectively. However, these viruses are quite common infections, so some susceptibility to the virus must be present to cause MND.

To see if humans with MND have a genetic susceptibility to metal toxins or viruses, we have set up a DNA bank with blood samples from people both with and without MND. This research is looking at the interactions between genes and environmental toxins or viruses that could underlie MND.

- Link To Laboratory -

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J Pollard
Demyelination and sensory neuropathies.

J Prineas
Multiple sclerosis and neural changes after lesions.

R Taylor
Myelination and developmental neural changes.

M Thomson • Go To Laboratory
Interactions of cytokines and steroids in Multiple Sclerosis

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