 |
|
Cure found for Huntington disease in mice offers hope for treatment in humans
June 16, 2006VANCOUVER, B.C.: Researchers at the University of British Columbia's Centre for Molecular Medicine and Therapeutics (CMMT) have provided ground-breaking evidence for a cure for Huntington disease in a mouse offering hope that this disease can be relieved in humans.
Published today in Cell journal, Dr. Michael Hayden and colleagues discovered that by preventing the cleavage of the mutant huntingtin protein responsible for Huntington disease (HD) in a mouse model, the degenerative symptoms underlying the illness do not appear and the mouse displays normal brain function. This is the first time that a cure for HD in mice has been successfully achieved.
"Ten years ago, we discovered that huntingtin is cleaved by 'molecular scissors' which led to the hypothesis that cleavage of huntingtin may play a key role in causing Huntington disease", said Dr. Michael Hayden, Director and Senior Scientist at the Child and Family Research Institute's Centre for Molecular Medicine and Therapeutics. Dr. Hayden is also a Canada Research Chair in Human Genetics and Molecular Medicine.
Now a decade later, this hypothesis has resulted in a landmark discovery. "This is a monumental effort that provides the most compelling evidence of this hypothesis to date", said Dr. Marian DiFiglia, Professor in Neurology, Massachusetts General Hospital, Harvard Medical School and one of the world's leading experts on Huntington disease. "Dr. Hayden and his team have shown in convincing fashion that many of the changes seen in HD patients can be erased in HD mice simply by engineering a mutation into the disease gene that prevents the protein from getting cleaved at a specific site".
To explore the role of cleavage, Dr. Hayden's team established an animal model of HD that replicated the key disease features seen in patients. A unique aspect of this particular animal model is that it embodied the human HD gene in exactly the same way seen in patients. This replication allowed researchers to examine the progression of HD symptoms including the inevitable cleavage of the mutant huntingtin protein. In the study, researchers confirmed that the deadly cleavage is caused by a key enzyme called caspase-6. By blocking the action of this target, they showed that the mouse did not develop any symptoms of Huntington disease.
Hayden's team is now trying to test this model of prevention in a mouse using drug inhibitors and then ultimately in humans. "Our findings are important because they tell us exactly what we need to do next", said Dr. Rona Graham, Post Doctoral Fellow at the CMMT and lead author in the study.
This work is also pivotal for the individuals and families affected by Huntington disease. "Patients of this disease should know that this is a research milestone for all and that this work brings the field closer to finding effective treatment for a devastating disorder", said Dr. DiFiglia.
The Huntington Society of Canada (HSC), a national network of volunteers and professionals united in the fight against HD, echoed this sentiment. "This ground-breaking research provides great hope for the Huntington community", said Don Lamont, the Society's CEO and Executive Director. "This research brings us closer to treatment and ultimately a cure".
Huntington disease is a degenerative brain disease that affects one in every 10,000 Canadians. One in 1,000 is touched by HD — for example, as a person with HD, a family member, a person at risk, caregiver or friend. The disease results from degeneration of neurons in certain areas of the brain causing uncontrolled movements, loss of intellectual faculties, and emotional disturbances. Currently, there is no treatment to delay or prevent HD in patients.
University of British Columbia
Now a decade later, this hypothesis has resulted in a landmark discovery. "This is a monumental effort that provides the most compelling evidence of this hypothesis to date", said Dr. Marian DiFiglia, Professor in Neurology, Massachusetts General Hospital, Harvard Medical School and one of the world's leading experts on Huntington disease. "Dr. Hayden and his team have shown in convincing fashion that many of the changes seen in HD patients can be erased in HD mice simply by engineering a mutation into the disease gene that prevents the protein from getting cleaved at a specific site".
To explore the role of cleavage, Dr. Hayden's team established an animal model of HD that replicated the key disease features seen in patients. A unique aspect of this particular animal model is that it embodied the human HD gene in exactly the same way seen in patients. This replication allowed researchers to examine the progression of HD symptoms including the inevitable cleavage of the mutant huntingtin protein. In the study, researchers confirmed that the deadly cleavage is caused by a key enzyme called caspase-6. By blocking the action of this target, they showed that the mouse did not develop any symptoms of Huntington disease.
Hayden's team is now trying to test this model of prevention in a mouse using drug inhibitors and then ultimately in humans. "Our findings are important because they tell us exactly what we need to do next", said Dr. Rona Graham, Post Doctoral Fellow at the CMMT and lead author in the study.
This work is also pivotal for the individuals and families affected by Huntington disease. "Patients of this disease should know that this is a research milestone for all and that this work brings the field closer to finding effective treatment for a devastating disorder", said Dr. DiFiglia.
The Huntington Society of Canada (HSC), a national network of volunteers and professionals united in the fight against HD, echoed this sentiment. "This ground-breaking research provides great hope for the Huntington community", said Don Lamont, the Society's CEO and Executive Director. "This research brings us closer to treatment and ultimately a cure".
Huntington disease is a degenerative brain disease that affects one in every 10,000 Canadians. One in 1,000 is touched by HD — for example, as a person with HD, a family member, a person at risk, caregiver or friend. The disease results from degeneration of neurons in certain areas of the brain causing uncontrolled movements, loss of intellectual faculties, and emotional disturbances. Currently, there is no treatment to delay or prevent HD in patients.
University of British Columbia
Huntington Disease Current Events and Huntington Disease News RSSResearchers estimate risk of transmission of Huntington's disease to offspring among male carriers
Researchers from Boston University School of Medicine (BUSM) have quantified the probability of a male who carries a "high normal" variant of the Huntington's Disease (HD) gene having a child who develops the disease.
New hope for treatment of neurodegenerative disorder
Researchers from the University of Southern California have taken an important first step toward protecting against Huntington disease using gene therapy.
Huntington disease begins to take hold early on
A global analysis of brain proteins over a 10-week period in a mouse model of Huntington Disease has revealed some new insights into this complex neurodegenerative disorder.
Stowers Institute's Workman Lab discovers novel histone demethylase protein complex
The Stowers Institute's Workman Lab has discovered a novel histone demethylase protein complex characterized in work published today in Molecular Cell.
Abnormal glutamine repeats interfere with key transcription factor, leading to neurodegeneration
Although repeating sequences of three nucleotides encoding some of the bodies' 20 amino acids are a normal part of protein composition, abnormal expansion of trinucleotide repeats is the known cause of multiple inherited neurodegenerative disorders, including Huntington disease.
Tufts University biologists link Huntington's disease to health benefits in young
For years researchers in neurology have believed that people with Huntington's disease have more children than the general population because of behavioral changes associated with the disease that lead to sexual promiscuity.
A new molecular zip code, and a new drug target for Huntington's disease
McMaster University researchers have first insight into how Huntington's disease (HD) is triggered. The research will be published online in the British Journal, Human Molecular Genetics, on Monday, August 20.
Elevated testosterone kills nerve cells
A Yale School of Medicine study shows for the first time that a high level of testosterone, such as that caused by the use of steroids to increase muscle mass or for replacement therapy, can lead to a catastrophic loss of brain cells.
More Huntington Disease Current Events and Huntington Disease News Articles
 |
Huntington's Disease (The Facts) by Oliver W J Quarrell (Author) Huntington's disease is a genetically inherited condition which results in severe nerve-cell damage in the brain. The hereditary and debilitative nature of the disease means that many people are involved either directly or indirectly by this condition. The recent identification of the faulty gene involved has made the diagnosis of this condition simpler. The majority of people develop the disease between the ages of 35 and 55 years, so for those aware of their genetic risk there are dilemmas to consider - should you have a test to see if you have the gene? Should you start a family? The new edition of this successful book specifically designed for families of patients with Huntington's disease has been expanded to include a number of important new developments in research and... |
 |
A New Promise by Julie Eller (Author) |
 |
Blue Institution by Ernie Kish (Author) Scott Porter, institutionalized at the age of twenty in a state institution for the developmentally delayed. Ironically, Scott is not developmentally delayed. He suffers from a hereditary neurological disease, Huntington's chorea, which Scott admits is "one of Mother Nature's cruelest jokes."Huntington's has rendered Scott virtually unable to do anything for himself, including communicating verbally. The only communication he has is the subtle vibrations in a person's being, that only a handful of the more sensitive care-givers, where he now lives, understand. Scott's brilliant mind is still intact, it's his body that doesn't want to function any-longer.So with all the problems that Scott has, how does he tell his story? "It's a miracle." Scott tells his story through his mind-his... |
 |
Learning to Live With Huntington's Disease: One Family's Story by Sandy Sulaiman (Author) Huntington's Disease (HD) is a hereditary illness passed on via a defective gene. There is a fifty per cent chance of inheriting it from a parent and there is yet no cure. "Learning to Live with Huntington's Disease" is one family's poignant story of coping with the symptoms, the diagnosis and the effects of HD. This book presents the struggles and strengths of the whole family when one member loses their future to a terminal illness. Told by the sufferer and other significant family members, the individuals describe the burden of watching yourself and others for symptoms of HD, including involuntary movements, depression, clumsiness, weight loss, slurred speech and sometimes violent tendencies. The family recounts the challenge to remain united and describes how they approached issues... |
 |
Huntington's Disease Awareness Ribbon Mouse Pad by MyHeritageWear.com The Huntington's Disease Ribbon proudly displayed on a mouse pad. There is no better way to achieve awareness for the meaning of the Huntington's Disease Ribbon than to display it on your mouse pad for everyone to see. The mouse pad measures at 9.25 x 7.75, it is machine washable, and the colors will not fade or run. Start gaining awareness today by presenting your Huntington's Disease Ribbon mouse pad at work or at home. It is certain to keep your mouse rolling in style all while gaining support and awareness! |
 |
Insurrection Also With: WB (Producer) |
|
Huntington's Disease [VHS] Directed By: Tina Campbell | |
 |
Huntington's Disease (Genes and Disease) by David M. Lawrence (Author) Huntington's disease, or Huntington's chorea, is a progressive genetic disease marked by death of brain cells coupled with loss of muscular control and coordination, declining mental abilities, and erratic behavior. Currently, this form of dementia has no cure. "Huntington's Disease" introduces this disease, detailing its history and progression, and discusses the search for the gene that causes it and the development of genetic tests for the gene. This title also addresses the ethical questions of testing people for a disease whose symptoms typically develop later in life. |
 |
Prevagen Aequorin, 30-Count Bottle by Prevagen PrevagenTM is a ground breaking dietary supplement from a jellyfish and is designed to fight aging. Scientific studies show a decrease in cellular death by up to 50 percent when treated with Prevagen. Prevagen is the first supplement to address aging through the restoration of calcium-binding proteins. Replenish age-fighting proteins, feel younger, and have more energy with Prevagen. You lose it, Prevagen Replaces it Throughout the aging process your body loses its ability to make calcium-binding proteins.* The lack of these protective proteins can result in the death of your cells that are involved in learning and memory. Prevagen is designed to replace these proteins and help you fight aging.* The animation to the left demonstrates how Prevagen can replenish... |
 |
Faces of Huntington's by Carmen Leal-Pock (Author) Faces of Huntington's is an incredible collection of stories, essays, poems, and quotes of those who are in some way connected to Huntington's Disease. The book focuses on over sixty people who have HD, are at risk, caregivers, other family members, adn friends. It is a beacon of light in what is often a dark world. 30,000 Americans have this terminal neurological disorder. An additional 150,000 are at-risk. Somehow, despite the horror of Huntington's Disease, there is loving support, a positive attitude, and unending hope. There are stories that make you rejoice at the unswerving faith and those that make you cry at the senseless loss. Faces of Huntington's gives readers a glance at the faces of heroes. It is guaranteed to bring tears of joy, hope, love and ... |
| © 2009 BrightSurf.com |