Huntington's News

Dantrolene protects neurons from Huntington's disease

Posted: 30th November 2011

Huntington's disease (HD) is characterized by ongoing destruction of specific neurons within the brain. It affects a person's ability to walk, talk, and think - leading to involuntary movement and loss of muscle co-ordination. New research published in BioMed Central's open access journal Molecular Neurodegeneration shows that the RyanR inhibitor Dantrolene is able to reduce the severity of walking and balance problems in a mouse model of HD.

Read the full story on the EurekAlert! website

Prana's Planned Huntington's Trial Receives Strong Interest From Patients and Researchers

Posted: 11th October 2011

Prana Biotechnology Limited received a very positive reaction from international patient groups and researchers at this week's World Congress on Huntington's Disease in Melbourne. The company had a major presence at the Congress and the Prana team has held numerous meetings and conversations with patient groups and researchers.

Read the full story on the Market Watch website.

Getting The Data Out - A New Scientific Journal Just For HD

Posted: 3rd July 2011

The search for better treatments for HD requires a lot of effort by researchers across the globe. Time is of the essence: the ideal time for a treatment for HD is yesterday. Data produced by all these researchers need to be made available sooner rather than later, so that others can build on what is already known. PloS is a new journal launched to speed up the process of scientific discovery in HD.

The traditional way to publish new findings is for researchers to send data with a ‘story’ to a scientific journal. A panel of experts then reviews that story, to judge whether the data and story are solid, and also whether the story is important enough to be interesting to the journal’s audience. This process is known as ‘peer review’.

This approach has advantages: it ensures that what is published is scientifically sound. Once published, articles can be retrieved via the internet sites like PubMed, and used by other scientists to guide and advance their own research.

However, there are several drawbacks, too. Firstly, the time between doing the research and the story being published can be very long. Sometimes the story has to be offered to several journals, one after the other, before it is eventually published. Several years can pass like this.

Secondly, most journals survive by selling copies, so they have to rate the importance of each story. Anything that might not be interesting to that journal’s audience is likely to be rejected, even if the story is scientifically sound.

That introduces bias to what’s available in the scientific literature. It favours exciting stories, but makes it difficult to publish solid, well-conducted scientific research if the results are less glamorous – for instance, if they show that a particular approach, idea or experiment has not helped. These are known as ‘negative results’.

Another problem is that a complete scientific ‘story’ might take five or ten years to research from start to finish. Along the way, interesting data might be produced, but because they don’t tell a complete story, they are unlikely to be published and seen by other researchers.

“Negative data”, or data that don’t make a complete story, can still be really useful to other researchers. For science to make progress, knowing what doesn’t work can be as useful as knowing what does.

Imagine ten researchers in different places, working on similar scientific projects, that could have been shown to be pointless already, if someone had published a single negative result. All that effort, time and money could have been put to much better use.

A new platform for publishing was launched in September 2010, aiming to make HD research more efficient by publishing the results that would otherwise never be seen, and shortening the time it takes to get data published.

This platform is called Public Library of Science (PloS) Current Huntington Disease and is supported by the CHDI Foundation.

The HD Outreach Service Has Moved

Posted: 3rd July 2011

The HD Outreach Service has now moved from Lottie Stewart Hospital at Dundas to Westmead Hospital. Please note their new contact details below:

Westmead Hospital HD Service,
Westmead Hospital,
Hawkesbury Road,
Westmead 2145
Phone: 02 98459960

Neutrons provide first sub-nanoscale snapshots of Huntington's disease protein

Posted: 20th May 2011

Researchers at the Department of Energy's Oak Ridge National Laboratory and the University of Tennessee have for the first time successfully characterized the earliest structural formation of the disease type of the protein that causes Huntington's disease. The incurable, hereditary neurological disorder is always fatal and affects one in 10,000 Americans.

Novel mouse model provides insight into rare neurodegenerative disease

Posted: 12th May 2011

New research sheds light on common pathogenic mechanisms shared by Huntington's disease (HD) and HD-like disorders. The study, published by Cell Press in the May 12, 2011, issue of the journal Neuron, uses a new transgenic mouse model for an HD-like disorder to unravel complex molecular events that drive disease pathology.

Read more about this research.

Huntington Society "like my child," Ariel Walker says

Posted: 12th May 2011

CAMBRIDGE — Ariel Walker only knew one person with Huntington’s disease when she started a national support organization with her husband, Ralph, almost 40 years ago. Since then, the Cambridge woman got to know many people affected by the fatal neurological disease and is determined to keep working to offer support and hopefully an effective treatment. Those people are the reason she stays involved with the Canadian charity.

Read the full story on The website.

Huntington's disease protein has broader effects on brain, study shows

Posted: 14th April 2011

In Huntington's disease, the mutant protein known as huntingtin leads to the degeneration of a part of the brain known as the basal ganglia, causing the motor disturbances that represent one of the most defining features of the fatal disease. But a new study reported in the April issue of Cell Metabolism, a Cell Press publication, shows that the mutant protein also is responsible for metabolic imbalances in the hypothalamus, a brain region that plays an important role in appetite control.

"This helps to explain metabolic changes and increases in appetite that have been observed in people at the early stages of disease," even before any motor symptoms appear, said Åsa Petersén of Lund University in Sweden. "It should encourage us to do more clinical studies. If we really understand the pathways that are affected, it may lead to new targets for intervention."

Read the full story on the EurekAlert! website.

UK Insurers Agree to Extend Ban on Using Genetic Tests

Posted: 14th April 2011

The Association of British Insurers announced the extension of an agreement under which the results of genetic testing will not be used to deny insurance to applicants.

The agreement, known as the Concordat and Moratorium on Genetics, will now run until 2017. It was introduced in 2001 after agreement between the industry and the U.K. Department of Health.

Like to know more? Visit

UMMS researchers develop new technology to screen and analyze genetic mutations

Posted: 14th April 2011

WORCESTER, Mass. – A single change to even one of the thousands of DNA codes that make up each gene in the human genome can result in severe diseases such as cancer, cystic fibrosis, muscular dystrophy or Huntington's Disease. A similarly minor change in the DNA of a virus or bacteria can give rise to drug resistant strains that are difficult for physicians to treat with standard drug therapies. For these reasons, scientists have long sought ways to study the effects genetic mutations can have on an organism but have been hampered in these efforts by an inability to easily and efficiently produce and analyze the thousands of potential changes possible in even one small gene.

A new study by scientists at the University of Massachusetts Medical School, published in Early Edition of the Proceedings of the National Academy of Sciences online on April 4, describes a novel technique to produce all potential individual mutations and using deep sequencing technology simultaneously analyze each change's impact on the cell.

More information on the EurekAlert! website.


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