The United States National Institutes of Health (NIH) have awarded Lincoln University a US$705,000 grant to continue research for another three years into a currently incurable childhood degenerative brain condition—Batten disease.
NIH funding is prestigious and difficult to obtain, particularly foreign grants which require a unique opportunity to lead research worldwide. The NIH have determined that the research being undertaken into Batten disease by Dr David Palmer of Lincoln University is ground breaking enough to meet this high standard.
Dr Palmer has been breeding and studying sheep with Batten disease for the last 25 years because their brains have a similar anatomy, physiology and genetic organisation to human brains. The development of the disease in sheep and humans is also similar, including the clinical course, symptoms and neuropathology.
Batten disease is actually a group of closely related diseases. They cause severe brain atrophy, blindness and seizures of increasing severity that lead to premature death. There are no effective therapies and even palliative care is difficult.
Dr Palmer has discovered neuro-inflammation plays a primary role in the development of the disease. These findings open up the possibility that control of cellular inflammation may be beneficial to patients, especially as an adjunct to gene or enzyme therapy.
The NIH funding will allow an international research team led by Dr Palmer, with collaborators in Auckland, Sydney, London and Helsinki, to further investigate the molecular defects in the affected sheep genes. The team will also study the relationship between neuro-inflammation, neuronal loss and the development of disease in affected sheep brains.
“This will allow more accurate targeting of therapeutic suppression. Starting with widely used anti-inflammatory drugs, trials will be conducted on the effects of pharmacological suppression of inflammation on the development of the disease.”
The research will also further define the process by which neurons are created to populate the growing brain. This process, called neurogenesis, is a normal event in brain development, and drops to almost nothing in the mature normal brain. In the brains of mature diseased sheep it continues, perhaps in a futile attempt to generate replacements for the neurons dying in the affected cortical regions.
As well as being important for Batten disease, the research findings may also have relevance to other neurodegenerative diseases such as Alzheimer’s disease, and other storage diseases such as Sandhoff disease.
Much of Dr Palmer’s work has been funded by a succession of NIH grants supplemented by money from the Neurological Foundation of New Zealand and the Batten Disease Support and Research Association (USA). The Canterbury Medical Research Foundation and Lysosomal Diseases New Zealand have also provided funding at critical times.
FOR FURTHER INFORMATION CONTACT
Elizabeth Owens, Marketing and Communications Manager
Lincoln University, Canterbury
Tel: 64 3 325 2811 ext 8655
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Email: Elizabeth Owens