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Issue 98, September 2007

Freemason Charity recipient receives
Young Scientist of the Year Award

Jessie Jacobsen, a PhD student in The University of Auckland's Department of Anatomy with Radiology has been named New Zealand's Young Scientist of the Year for 2007 for her work on Huntington's disease. Jessie is one of four University of Auckland students chosen as outstanding scientists in this year's MacDiarmid awards from the 120 entries into the award competition. The awards ceremony was held in Auckland on Wednesday 20th June 2007

With the support of Freemasons New Zealand, Jessie's PhD looks at the progression of Huntington's disease in Sheep.

Understanding how Huntington's disease develops in a large animal with a similar brain structure to humans could give scientists some insight into how the disease develops in the human brain. In turn, this knowledge will assist in the development and testing of new treatments for Huntington's and other neurological disorders.

Freemasons New Zealand has supported the transgenic Huntington's disease project led by New Zealand's pre-eminent neuroscientist, Professor Richard Faull, from the very outset. Professor Faull comments:

"The Faculty of Medical and Health Sciences is hugely indebted to the Freemasons of New Zealand for their generous and unstinting support of this research. We are really starting to make progress now and this honour for Jessie is wonderful recognition not only of Jessie's ability but of the loyalty and dedication of our funding partners, the Freemasons."

The MacDiarmid Young Scientists of the Year Awards are presented by the Foundation of Research, Science and Technology and are designed to recognize excellent research, science and technology while also promoting the importance of good science communication. As winner of the MacDiarmid Young Scientist of the Year title, Jessie Jacobsen will receive a trip to the UK's British Association Festival of Science along with a $10,000 grant.

"The MacDiarmid Awards recognises the achievements of our up and coming scientists, and is an excellent vehicle to highlight the dedication and commitment of our students," says Professor Tom Barnes, Deputy Vice Chancellor (Research) at The University of Auckland. "The University is proud to once again be the home of the Young Scientist of the Year."

Below is an article that Jessie has written around her research and provides a brief look into the trauma of living with Huntington's disease as well as the research itself.


Jessie Jacobsen

Often I lie awake at night and count sheep, not to put me to sleep, but in the hope that one of them might help Mary lead a life that's not so cruelly defined by a gene.

I met Mary two years ago, with tears streaming down her face she told me her story. Twenty five years ago, she met 'Mr. Right'; they got married and had four adorable kids. The first fifteen years went swimmingly, then she started noticing some odd behaviour in her husband, a twitch here and there, a stumble when he started to walk. When he began having unpredictable flashes of anger she started to worry and then the neighbours embarrassingly assumed that her husband's unsteady walk was because he was constantly drunk, she decided to take him to the doctor. The diagnosis he received was devastating. It would affect not only her husband but the whole family. He had Huntington's disease (HD), a late onset fatal brain disorder for which there is currently no cure or treatment.

The gene that would kill her husband sits in the short arm of everyone's fourth chromosome. It contains a region where the letters of the genetic alphabet C-A-G repeat up to ~35 times in the normal population. Those with greater than ~36 repeats of this sequence develop HD. Mary's husband had ~43 repeats. Because HD is dominantly inherited, their children has a 50/50 chance of inheriting the disease gene and if they had the disease or endure the torture, and hope, of not knowing. Her children decided to get tested.

Nature had dealt a harsh blow: all four had inherited their father's expanded repeat. Mary's husband has now died from HD and she is looking after her children, helping them cope with their nightmares; "Will I be able to get a job?" "Will anyone ever marry me?" "Should I have kids and risk passing the disease on to them?" She waits for this incurable brain disorder to ravage her children. So I think of Mary when I count sheep, six sheep in particular - Huzi, Rocket, Kiwi, Aussie, Taffie, and Kerry.

Over the last three years I have been generating a sheep model of HD in an effort to fast track a cure or therapy for this disease. Virtually all medical knowledge and treatment over the last century has involved animal models. Treatments for cancer, heart disease and vaccinations are all testament to the benefits of animal models in research, and I want to add HD to the list. So why sheep? Surprisingly, their brain structure is remarkably similar to humans (obviously there's more to eating grass than we think!) Also, because of their longevity (10-15 years) we can mimic the late onset of HD, giving us a window, before the symptoms start, in which we can trial drugs to try and halt the disease.

So how did we go about it? In the laboratory we generated the transgene, a piece of human DNA containing the gene which cause's HD. Using this transgene, colleagues at the South Australian Research and Development Institute used In Vitro Fertilization (IVF) to inject this piece of DNA into an egg of a ewe. The egg was then fertilized with sperm from a ram and the fertilized egg transferred back into the uterus of the ewe. Five months later we had the first HD lambing season in the world, with six of the lambs testing positive for the insertion of our transgene. We named them Huzi, Rocket, Kiwi, Aussie. Taffie and Kerry.

We have bred from the two female transgenic (Kiwi and Taffy) and call their offspring the F1 generation.

So why am I excited about this model? The answer to that question is what I'm seeing under the microscope. I have started looking in a region deep in the brain (the basal ganglia) of one of Kiwi's F1 offspring. This region is important in our control of mood and movement and is the first and most affected region in HD (hence Mary's husband's apparent 'drunken' movement and behaviour).

Receptors on cells (which receive and send information), normally present in regions of the basal ganglia, are reduced in this HD sheep, which is what happens in the human disease. These missing receptors cause a breakdown in communication in the brain. Regions which usually receive this information released from these missing receptors are becoming more active, trying to find the missing lines of communication, which is also seen in this HD sheep brain. If this model continues to show similar patterns of receptor changes and cell loss seen in the human disease, it will enable us to help find drugs that halt its destructive path, giving people like Mary and her family hope that their lives will not be forever tormented by this cruel disease.

So this is why I count sheep. I hope one day these sheep will help families like Mary's sleep peacefully, free of the nightmares they currently have to live with. So start counting, who knows when a sheep like Kiwi might change your life

Pictured with the lambs, Dr Russell Snell,Prof. Richard Faull and Jessie Jacobsen
Pictured with the lambs, Dr Russell Snell,
Prof. Richard Faull and Jessie Jacobsen

Acknowledgment: New Zealand Freemason Issue 2, 2007 (Vol 35)

Extra items of interest from the Internet

TV3 News report (Video on Demand 176)
An Auckland student has scooped New Zealand's Scientist of the Year award for her work in finding a cure for Huntington's disease. (Duration 1 minute 50 seconds.)

Listen to Jessie talking about her research (Radio NZ)
Jessie Jacobsen is the 2007 MacDiarmid Young Scientist of the Year for her innovative research on Huntington's disease. (Duration: 12 minutes approx.)

University of Auckland podcast interview
Jessie Jacobsen PhD, Department of Anatomy with Radiology, discusses her work on Huntington's disease that won her the MacDiarmid Young Scientist of the Year award. (Duration: 10 minutes approx.)

More photos ...
At the awards ceremony with minister Steve Maharey Foundation Chief Executive Murray Bain with MacDiarmid Young Scientist of the Year Jessie Jacobsen, runner up James Russell and Steve Maharey Minister for Research, Science and Technology At the awards ceremony Jessie Jacobsen and Dr Shieak Tzeng at the 2007 MacDiarmid Young Scientist Awards
PhD student Jessie Jacobsen with a section of brain affected by Huntington's disease (top) and a healthy sample below. Photo / Kenny Rodger (NZ Herald)

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