CBR Hosts Launch of ‘Minds for Minds’ Campaign

The Centre for Brain Research was the setting last week for the launch of the ‘Minds for Minds’ campaign, an initiative of the Autism Research Network New Zealand (ARNNZ), to raise awareness and funding for research into the genetic causes of Autism.  They are also seeking people on the autism spectrum to join a research register, which will be used to collect genetic and autobiographical information.

Over a hundred people packed our seminar room space to hear a series of the lead researchers from the network give a precis of their research, and how it contributes to the ‘overall picture’, before the unveiling of the campaign phrase ‘Minds for Minds’, together with its appealing brain logo.  To read columnist Deborah Hill Cone’s view of the campaign and its significance, click here:

ARNNZ lead researcher Professor Russell Snell with journalist Deborah Hill Cone.

ARNNZ lead researcher Professor Russell Snell with journalist Deborah Hill Cone.

Several of the founding members of ARNNZ are also CBR members, and it looks as though they mean to apply the same principles of working alongside clinical experts and members of the community to ensure the best results for those living with autism, and their families.  Go to www.arnnz.org to find out how you can be part of the picture.

ARNNZ members flank the 'Minds for Minds' logo. L-R: Associate Professor Karen Waldie (CBR), Dr Johanna Montgomery (CBR), Professor Ian Kirk (CBR), Dr Mike Taylor, Professor Russell Snell (CBR), Dr Jessie Jacobsen (CBR), and Dr Rosamund Hill

ARNNZ members flank the ‘Minds for Minds’ logo. L-R: Associate Professor Karen Waldie (CBR), Dr Johanna Montgomery (CBR), Professor Ian Kirk (CBR), Dr Mike Taylor, Professor Russell Snell (CBR), Dr Jessie Jacobsen (CBR), and Dr Rosamund Hill

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CBR in the News!

The Centre for Brain Research has been getting in the news this week – for all the right reasons!

On Wednesday 17th July, Professor Richard Faull was interviewed by Kathryn Ryan on Radio New Zealand’s ‘Nine to Noon’ programme, discussing the discovery of stem cells within the brain, and the controversy this finding caused in the research world.  They also cover the evolution of the Neurological Foundation Human Brain Bank, and the links it creates and maintains with clinician, community, and the families of those affected by neurological disease.  You can listen to a recording of the interview here: http://www.radionz.co.nz/audio/player/2562370

Later in the day, Professor Faull also featured on on Sky Sports’ ‘Deaker on Sport’ programme, talking with Murray Deaker and neurologist Rosamund Hill about concussion and our growing understanding of its long-term effects – always a relevant issue in our sport-focused nation.

A breakthrough in understanding the behaviour of stem cells as they ‘bed down’ in their destination within the brain has lead to media exposure for Dr Maurice Curtis and his lab, with a report on their latest research appearing in the New Zealand Herald.   Read the full article here: http://www.nzherald.co.nz/science/news/article.cfm?c_id=82&objectid=10899554

New Zealand’s top Huntington’s disease researchers to speak at national conference

New Zealand’s foremost Huntington’s Disease (HD) research and clinical experts will speak at the national associations’ conference in Auckland on Saturday 18 and Sunday 19 May.

The speaker line-up includes New Zealand’s top neuroscientist Distinguished Professor Richard Faull, an internationally recognised expert on neurodegenerative diseases of the human brain. Professor Faull is the Director of The University of Auckland’s Centre for Brain Research which has had an established HD research programme since 1981. Professor Faull’s presentation is titled Huntington’s Disease – Exciting Human Brain Research with Family Support. Fellow University of Auckland researcher Professor Russell Snell, a member of the global team that discovered the HD gene in 1993, will provide an overview of learnings about HD from a genetics perspective.

Neurologist and HD clinical specialist Dr Richard Roxburgh will discuss the progress of an international clinical trial called CREST E* which is in phase 3 and involves 650 participants from around the world. Neuropsychiatrist and fellow clinical specialist Dr Greg Finucane will outline drug therapies in his lecture titled Calming the Stormy Seas. Patient service and care updates will be provided by national Huntington’s Disease Association staff, and all presentations within the two-day speaker programme are designed for the general public to understand.

Professor Faull, who is patron of Huntington’s Disease Association Auckland, says “The conference will be an opportunity for patients, families and medical professionals to learn about new global and local research and the latest developments in prospective treatments. It will also be a unique opportunity for the HD community to share knowledge and learn from the experience of others. This will be a very special meeting of those who share the ultimate goal of improving patients’ lives and ending Huntington’s Disease. “

For further details about the conference and to register, please phone: (09) 815 9703 or email conference2013@hdauckland.org.nz 

The conference will be held at the Waipuna Hotel and Conference Centre in Auckland and is supported by the Centre for Brain Research, the Neurological Foundation of New Zealand, and the Lion Foundation.

 

 

Symptoms in genetic brain disease are reflected in brain cell loss

Scientists have wrestled to understand why Huntington’s disease, which is caused by a single gene mutation, can produce such variable symptoms.

An authoritative review by a group of leading experts summarizes the progress relating cell loss in the striatum and cerebral cortex to symptom profile in Huntington’s disease, suggesting a possible direction for developing targeted therapies. The article is published in the latest issue of the Journal of Huntington’s Disease.

Huntington’s disease (HD) is an inherited progressive neurological disorder for which there is presently no cure. It is caused by a dominant mutation in the HD gene leading to expression of mutant huntingtin (HTT) protein. Expression of mutant HTT causes subtle changes in cellular functions, which ultimately results in jerking, uncontrollable movements, progressive psychiatric difficulties, and loss of mental abilities.

Although it is caused by a single gene, there are major variations in the symptoms of HD. The pattern of symptoms shown by each individual during the course of the disease can differ considerably and present as varying degrees of movement disturbances, cognitive decline, and mood and behavioral changes. Disease duration is typically between ten and twenty years.

 Recent investigations have focused on what the presence of the defective gene does to various structures in the brain and understanding the relationship between changes in the brain and the variability in symptom profiles in Huntington’s disease.

 Analyses of post-mortem human HD tissue from the Human Brain Bank suggest that the variation in clinical symptoms in HD is strongly associated with the variable pattern of neurodegeneration in two major regions of the brain, the striatum and the cerebral cortex. The neurodegeneration of the striatum generally follows an ordered and topographical distribution, but comparison of post-mortem human HD tissue and in vivo neuroimaging techniques reveal that the disease produces a striking bilateral atrophy of the striatum, which in these recent studies has been found to be highly variable.

 “What is especially interesting is that recent findings suggest that the pattern of striatal cell death shows regional differences between cases in the functionally and neurochemically distinct striosomal and matrix compartments of the striatum which correspond with symptom variation,” says author Professor Richard Faull, Director of the Centre for Brain Research, University of Auckland.

 “Our own recent detailed quantitative study using stereological cell counting in the post-mortem human HD cortex has complemented and expanded the neuroimaging studies by providing a cortical cellular basis of symptom heterogeneity in HD,” continues Dr Faull. “In particular, HD cases which were dominated by motor dysfunction showed a major total cell loss (28% loss) in the primary motor cortex but no cell loss in the limbic cingulate cortex, whereas cases where mood symptoms predominated showed a total of 54% neuronal loss in the limbic cingulate cortex but no cell loss in the motor cortex. This suggests that the variable neuronal loss and alterations in the circuitry of the primary motor cortex and anterior cingulate cortex associated with the variable compartmental pattern of cell degeneration in the striatum contribute to the differential impairments of motor and mood functions in HD.”

The authors including Dr Henry Waldvogel note that there are still questions to be answered in the field of HD pathology, such as, how and when pathological neuronal loss occurs; whether the progressive loss of neurons in the striatum is the primary process or is consequential to cortical cell dysfunction; and how these changes relate to symptom profiles.

Professor Faull adds; “What is clear however is that the diverse symptoms of HD patients appear to relate to the heterogeneity of cell loss in both the striatum and cerebral cortex,” the authors conclude. “While there is currently no cure, this contemporary evidence suggests that possible genetic therapies aimed at HD gene silencing should be directed towards intervention at both the cerebral cortex and the striatum in the human brain. This poses challenging problems requiring the application of gene silencing therapies to quite widespread regions of the forebrain which may be assisted via CSF delivery systems using gene suppression agents that cross the CSF/brain barrier.”

Genetic research hits the global headlines

Research from the CBR has made the international news with advances in genetic therapies.

The transgenic Huntington’s sheep developed by Professors Richard Faull and Russell Snell has been featured in the prestigious magazine Scientific American. The article highlights how the sheep model was developed over many years of painstaking research, supported by the Freemasons of New Zealand.

Now 5 years on, the sheep is being used to study Huntington’s disease, which causes devastating movement and psychological symptoms which get worse as patients age. The sheep will hopefully be used to develop new drugs for this debilitating disease. The article can be viewed here.

Research into another genetic disease, Canavan disease, has also been published in the top-class journal Science Translational Medicine. The gene therapy breakthrough was developed by Associate Professor Debbie Young and Professor Matt During’s team, who research viral vectors as a way to alter disease progression in humans.

Canavan disease is a rare hereditary disorder which affects children, causing their entire nervous system to stop working. Long-term follow-up after the gene therapy has shown that patients had less seizures and slower brain shrinkage. The exciting research may mean that the children have better health as they grow up.

These important clinical developments both came about through painstaking preclinical research, and highlight just how important support for basic research is. You can read more about the need for basic research here.