Regulatory enzyme overexpression may protect against neurodegeneration in Huntington's disease. Treatment that increases brain levels of an important regulatory enzyme may slow the loss of brain cells that characterizes Huntington's disease (HD) and other neurodegenerative disorders. In a report receiving advance online publication in Nature Medicine, a Massachusetts General Hospital (MGH)-based research team reports that increased expression of Sirt1, one of a family of enzymes called sirtuins, in the brain of a mouse model of HD protected against neurodegeneration. They also identified a potential mechanism for this protective effect. "Diseases such as Huntington's, Parkinson's and Alzheimer's disease have different causative factors, but they share common themes – such as aggregation of misfolded proteins – and a unifying endpoint, the degenerative loss of neurons," says Dimitri Krainc, MD, PhD, of the MassGeneral Institute for Neurodegenerative Disease (MGH-MIND), the study's senior author.
Mood Stabilizers Offer Hope For Huntington’s Treatment. A protocol for studying the combined use of lithium and valproate in Huntington’s subjects was approved by the National Institutes of Health (NIH), Chuang pointed out, and he would have been a co-investigator. But the trial did not get off the ground because the principal investigator left NIH. In any event, in light of these new findings, “I very much hope that some clinicians will be interested in conducting a trial using both lithium and valproate for Huntington’s,” he said. Meanwhile, findings from a few small, short clinical trials do suggest that lithium or valproate might benefit Huntington’s patients.
For example, a 1973 trial found that lithium treatment strikingly reduced chorea and markedly improved voluntary movements in Huntington’s subjects. And a 2000 trial found that valproate helped relieve both movement disorders and psychosis in the subjects. The research was funded by the Intramural Research Program of the National Institute of Mental Health. Study Identifies Most Effective Ways To Assess Progression In Huntington's Disease, Which Could Speed Up Development Of Disease-modifying Drugs.
Biomarkers of Huntington’s Disease Progression ID’d. The effect sizes of detectable Huntington's disease-related changes over 24 months can identify potential outcome measures for assessing disease progression in therapeutic trials, according to a study published online Dec. 2 in The Lancet Neurology. FRIDAY, Dec. 2 (HealthDay News) -- The effect sizes of detectable Huntington's disease (HD)-related changes over 24 months can identify potential outcome measures for assessing HD progression in therapeutic trials, according to a study published online Dec. 2 in The Lancet Neurology.
Sarah J. Tabrizi, M.D., Ph.D., from the University College London, and colleagues used a prospective observational biomarker study to identify potential outcome measures for therapeutic trials. Longitudinal data were collected at baseline, 12, and 24 months from 117 individuals without HD but carrying the mutant HTT gene (premanifest HD), 116 participants with early HD, and 116 controls. Copyright © 2011 HealthDay. Submit your opinion: Dantrolene protects neurons from Huntington's disease. Public release date: 24-Nov-2011 [ Print | E-mail Share ] [ Close Window ] Contact: Dr Hilary Gloverhilary.glover@biomedcentral.com 44-020-319-22370BioMed Central 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. Progressive damage to medium spiny neurons (MSN) in the brain of a person with HD is responsible for many of the symptoms and is caused by an inherited recessive mutation in the gene 'Huntingtin'. Researchers from the University of Texas Southwestern Medical Center tested Dantrolene, a muscle relaxant which works by stabilizing calcium signaling, and showed that this drug could prevent calcium-dependent toxicity in laboratory grown neurons.
Dr Bezprozvanny explained, "One of the features of HD mice is the progressive loss of their NeuN-positive neurons. Notes to Editors 1. 2. 3. . [ Print | E-mail. Scientists identify protein form linked to Huntington's disease. Scientists at the Gladstone Institutes have discovered how a form of the protein linked to Huntington's disease influences the timing and severity of its symptoms, offering new avenues for treating not only this disease, but also a variety of similar conditions.
In a paper being published today in Nature Chemical Biology, the laboratory of Gladstone Senior Investigator Steven Finkbeiner, MD, PhD, singles out one form of a misfolded protein in neurons that best predicts whether the neuron will die. Neuronal death is key to the development of Huntington's symptoms—including erratic behavior, memory loss and involuntary muscle movement. This research underscores the value of the cross-disciplinary work done at Gladstone—a leading and independent biomedical–research organization—while revealing techniques that scientists anywhere can apply to conditions involving misfolded proteins, such as Alzheimer's disease and type 1 diabetes.
"Dr. Melatonin delays onset, reduces deaths in mouse model of Huntington's disease. Melatonin, best known for its role in sleep regulation, delayed the onset of symptoms and reduced mortality in a mouse model of Huntington's disease, say researchers at the University of Pittsburgh School of Medicine and Harvard Medical School. Their findings, published today in the Journal of Neuroscience, show for the first time that certain receptors for the hormone reside in the mitochondria, and that there are fewer of them both in affected mice and human brains.
Huntington's disease (HD) is an inherited, lethal disorder of involuntary movement, progressive loss of intellectual function and emotional problems, explained senior investigator Robert M. Friedlander, M.D., chair, Department of Neurological Surgery and UPMC Endowed Professor of neurosurgery and neurobiology.
A mutant protein, called huntingtin, kills neurons in the brain's striatum and then the cortex. New insight into the cellular defects in Huntington's disease. Huntington disease is a devastating neurogenerative disorder that causes a progressive loss of functional capacity and reduced life span. It is an inherited condition caused by a mutant HTT gene. Although this has been known for many years, the functions of the normal Htt protein and the mechanisms by which the mutant protein generated from the mutant HTT gene causes disease are not well understood.
A team of researchers led by Frédéric Saudou, at the Institut Curie, France, has now uncovered a new function for normal Htt protein and determined that this function is disrupted in a mouse model of Huntington disease and in patients with the disorder. Detailed analysis by Saudou and colleagues determined that normal Htt protein regulates the formation of cellular structures known as cilia and that cilia were longer and disorganized in the mouse model of Huntington disease and patients.
Study finds transcriptional biomarker for Huntington's disease. Huntington's disease, a devastating genetic disorder that causes degeneration of nerve cells in the brain, affects more than 15,000 Americans, and at least 150,000 are at risk of developing the disease. There is no known cure or treatment for the disease -- which starts with changes in mood, judgment, memory, and other cognitive functions and inevitably leads to increasing physical disability, dementia and death.
In a new research paper published in the Proceedings of the National Academy of Sciences early edition online, researchers identify a transcriptional biomarker that may assist in the monitoring of disease activity and in the evaluation of new medications. Researchers analyzed 119 human blood samples from both Huntington's disease patients and others.
They found that individuals with Huntington's disease overexpressed the gene, H2AFY, in their blood. "We know how to diagnose HD. Neutrons provide first sub-nanoscale snapshots of Huntington's disease protein. 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. Huntington's disease is caused by a renegade protein "huntingtin" that destroys neurons in areas of the brain concerned with the emotions, intellect and movement.
All humans have the normal huntingtin protein, which is known to be essential to human life, although its true biological functions remain unclear. "Bio-SANS is a great instrument for taking time-resolved snapshots. You can look at how this stuff changes as a function of time and be able to catch the structures at the earliest of times," Stanley said. Normal huntingtin contains a region of 10 to 20 glutamine amino acids in succession. "That is the future hope. Huntington's disease breakthrough: New research offers promise of medical intervention. Medical researchers may have uncovered a novel approach to treat an incurable and ultimately fatal neurodegenerative disease that affects hundreds of thousands of people.
Two international studies, one led by the University of Leicester, and the other a collaboration with Leicester led by scientists in the USA, hold out promise for slowing down the development of Huntington's disease -- and potentially, Alzheimer's and Parkinson's diseases. The research, which is in its early stages, represents an important milestone in understanding these debilitating conditions. Huntington's disease is a devastating inherited neurodegenerative disorder that is always fatal. The disorder of the central nervous system causes progressive degeneration of cells in the brain, slowly impairing a person's ability to walk, think, talk and reason. Approximately 1 in 10,000 individuals are affected worldwide. Natural compound shows promise against Huntington's disease. Fisetin, a naturally occurring compound found in strawberries and other fruits and vegetables, slows the onset of motor problems and delays death in three models of Huntington's disease, according to researchers at the Salk Institute for Biological Studies.
The study, published in the online edition of Human Molecular Genetics, sets the stage for further investigations into fisetin's neuroprotective properties in Huntington's and other neurodegenerative conditions. Huntington's disease (HD) is an inherited disorder that destroys neurons in certain parts of the brain and slowly erodes victims' ability to walk, talk and reason. It is caused by a kind of genetic stutter, which leads to the expansion of a trinucleotide repeat in the huntingtin protein. When the length of the repeated section reaches a certain threshold, the bearer will develop Huntington's disease. In fact, the longer the repeat, the earlier symptoms develop and the greater their severity.