Huntington's Disease Press Releases
Scientists adopt new strategy to find Huntington’s disease therapies
Friday, Aug 7, 2015
Scientists searched the chromosomes of more than 4,000 Huntington’s disease patients and found that DNA repair genes may determine when the neurological symptoms begin. Partially funded by the National Institutes of Health, the results may provide a guide for discovering new treatments for Huntington’s disease and a roadmap for studying other neurological disorders.
NIH Names Walter J. Koroshetz, M.D. Director of the National Institute of Neurological Disorders and Stroke
Thursday, Jun 11, 2015
National Institutes of Health Director Francis S. Collins, M.D., Ph.D. announced today the selection of Walter J. Koroshetz, M.D., as the Director of the National Institute of Neurological Disorders and Stroke (NINDS). He has served as Acting Director of the NINDS since October, 2014.
Scientists map memorable tunes in the rat brain
Tuesday, Mar 3, 2015
Lights, sound, action: we are constantly learning how to incorporate outside sensations into our reactions in specific situations. In a new study, brain scientists have mapped changes in communication between nerve cells as rats learned to make specific decisions in response to particular sounds.
3-D changes in DNA may lead to a genetic form of Lou Gehrig’s disease
Wednesday, Mar 5, 2014
New findings reveal how a mutation, a change in the genetic code that causes neurodegeneration, alters the shape of DNA, making cells more vulnerable to stress and more likely to die.
For a healthy brain, don’t let the trash pile up
Sunday, Jul 21, 2013
Recycling is not only good for the environment, it’s good for the brain. A study using rat cells indicates that quickly clearing out defective proteins in the brain may prevent loss of brain cells.
Of Mice and Flies: A Cutting-Edge Method for Detecting Neurodegenerative Disease Targets
Thursday, May 16, 2013
Studying neurodegenerative diseases can be like investigating a crime. Scientists inspect damaged nervous tissue, or “the scene”, for suspicious molecules and then work backwards to explain how the suspects may have killed nerve cells. Recently two research groups, one in the United States and the other in the United Kingdom, collaborated to develop a new way to quickly round up many more suspects and test their “alibis”. Their results may lead to more effective treatments for Alzheimer’s disease, Parkinson’s disease and a variety of other neurodegenerative disorders.
Breaking News from Society for Neuroscience 2012
Wednesday, Oct 17, 2012
Hundreds of NIH-funded studies are being presented at the 2012 Society for Neuroscience annual meeting. Here, the National Institute of Neurological Disorders and Stroke has highlighted a selection of studies and events led by our grantees.
Patient-derived stem cells could improve drug research for Parkinson's
Wednesday, Jul 4, 2012
Researchers have taken a step toward better drug therapies for Parkinson's disease and Huntington's disease by investigating signs of distress and vulnerability in patient-derived cells. Cells derived from patients with Parkinson's had different responses to drug treatments depending on the type of Parkinson's each patient had. These are the latest advances from the NINDS iPS cell consortia.
‘Anti-aging’ genes have anti-Huntington’s effects in mice
Monday, Apr 30, 2012
Sirtuins have been implicated in promoting longer life, but recently some scientists have begun to think that the genes influence the susceptibility to age-related diseases, rather than the aging process itself. Now two studies have found that boosting the levels of the sirtuin-1 gene reduces the loss of brain tissue in mouse models of Huntington’s disease.
Induced Pluripotent Stem Cells Give Investigators a New Window into Neurological Disease
Friday, Jun 24, 2011
It is possible to take a sample of skin cells and induce them to behave like embryonic stem cells. Scientists are using these so-called induced pluripotent stem (iPS) cells to study disease mechanisms and test potential therapeutic drugs. In 2009, NINDS funded three consortia to develop iPS cell lines from individuals with Parkinson’s disease, amyotrophic lateral sclerosis (ALS), and Huntington’s disease.
A Mitochondrial Meltdown in Huntington's Disease?
Friday, Jun 17, 2011
A study in Nature Medicine shows that the cellular energy factories known as mitochondria become fragmented in cells affected by Huntington's disease. Normal mitochondria have a dynamic structure, fusing together and splitting apart, but in Huntington's, an enzyme called DRP1 may shift the balance toward splitting. The enzyme could be a target for therapy.
National Advisory Neurological Disorders and Stroke Council Welcomes Six New Members
Thursday, Feb 3, 2011
The NINDS announced that six new members have joined its National Advisory Neurological Disorders and Stroke Council, the Institute’s principal advisory body regarding research program planning and priorities. The new members are Ben A. Barres, PhD, Robert B. Darnell, MD, PhD, Sharon E. Hesterlee, PhD, Eve Esther Marder, PhD, Robert Enrico Pacifici, PhD, and Amita Sehgal, PhD.
Spinocerebellar Ataxia 2 Gene is a Risk Gene for ALS
Friday, Nov 26, 2010
A recent study links sporadic ALS to a stutter-like mutation in the ataxin-2 gene. This same kind of mutation is known to be the culprit in another rare neurological disease, spinocerebellar ataxia type 2 (SCA2). “Having an expansion in the ataxin-2 gene does not necessarily cause ALS, but it does increase the risk for it,” said Aaron Gitler, Ph.D, a cell biologist at the University of Pennsylvania School of Medicine.
Therapeutic Strategy for Huntington’s Disease Could Focus on Containing Cell ‘Shrapnel’
Wednesday, Nov 24, 2010
Writing in the journal Neuron,* investigators report that enzymes involved in ailments from stroke to cancer also play a role in Huntington’s disease. Blocking the function of these enzymes, called matrix metalloproteinases (MMPs), is beneficial in cell and animal models of Huntington’s, and could be an effective therapeutic strategy for patients.
X-Rays Reveal 3-D Structural Image of Brain Receptor
Wednesday, Nov 10, 2010
Researchers led by Eric Gouaux at Oregon Health and Science University have built a three-dimensional image of a glutamate receptor, a workhorse protein of brain communications. The scientists uncovered the receptor’s form by bombarding it with X-rays – a technology called X-ray crystallography. The findings are expected to yield new insights into receptors and their critical role in thinking, learning and memory.
Small Changes in Protein Chemistry Play Large Role in Huntington’s Disease
Thursday, Dec 24, 2009
In Huntington’s disease, a mutated protein in the body becomes toxic to brain cells. Recent studies have demonstrated that a small region adjacent to the mutated segment plays a major role in the toxicity. Two new studies supported by the National Institutes of Health show that very slight changes to this region can eliminate signs of Huntington’s disease in mice.
New Route Identified for Clearing Away the Toxic Protein that Causes Huntington’s Disease
Wednesday, Jun 17, 2009
In Huntington’s disease (HD), a toxic protein accumulates inside brain cells, leading to symptoms such as uncontrolled movements, impaired thinking and personality changes. Researchers have now identified a chemical tag that attaches to this protein, sends it through a cellular waste handling system and prevents its harmful effects. Efforts are underway to identify drugs that could stimulate tagging of the protein and thus slow the course of HD.
Four New Members Named to National Neurology Advisory Council
Thursday, Sep 18, 2008
The National Institute of Neurological Disorders and Stroke (NINDS) has appointed four new members to its major advisory panel, the National Advisory Neurological Disorders and Stroke Council. The NINDS, a component of the National Institutes of Health (NIH), is the nation's primary supporter of basic, translational, and clinical research on the brain and nervous system. NINDS Director Story Landis, Ph.D., formally introduced the new members, who will serve through July 2012, at the Council's September 18, meeting.
Huntington’s Disease Protein Affects Nerve Signaling; Study Suggests New Treatments
Thursday, Jun 26, 2008
The abnormal protein found in Huntington’s disease (HD) leads to an unusually large amount of nerve signaling early in the disease process, before other problems appear, a new study shows. Partially blocking these nerve signals prevents neuron death and loss of motor function in fruit flies models of HD. The findings suggest possible new ways of delaying the onset or slowing the progression of the disease.
Researchers Develop First Transgenic Monkey Model of Huntington’s Disease
Friday, May 23, 2008
Scientists have developed the first genetically altered monkey model that replicates some symptoms observed in patients with Huntington's disease. This advance, reported in Nature, could lead to major breakthroughs in the effort to develop new treatments for a range of neurological diseases.
Study Suggests Idebenone May Improve Neurological Function in Friedreich's Ataxia
Wednesday, Dec 5, 2007
Results of a placebo-controlled, double-blind phase II study of the antioxidant idebenone in children with Friedreich's ataxia (FA) suggest that the treatment may lead to improvements in neurological function. It is the first placebo-controlled study to suggest that the neurological deterioration associated with this disease can be slowed or reversed.
Lithium May Offer Relief from Rare but Devastating Neurological Disorders
Thursday, Aug 2, 2007
Lithium carbonate, a compound commonly used to treat depression, might also provide symptomatic relief for a group of inherited movement disorders that includes the fatal disease spinocerebellar ataxia type 1 (SCA1).
Therapeutics for Huntington's and Related Diseases Could Pack a One-Two Punch
Tuesday, Jun 5, 2007
Added to its devastating neurological symptoms, Huntington's disease (HD) carries with it a lesser-known horror. The genetic mutation that causes the disease can grow larger, causing its symptoms – involuntary movements, dementia, and dramatic personality changes – to grow worse across generations and even during a single lifetime. New research sheds light on how the mutation grows and offers hope for locking it down.
NINDS Names Dr. Walter Koroshetz as Deputy Director
Wednesday, Jan 3, 2007
The National Institute of Neurological Disorders and Stroke (NINDS), part of the National Institutes of Health (NIH), has named Walter J. Koroshetz, M.D., as its Deputy Director. Effective January 2, 2007, he will work with the NINDS Director in program planning and budgeting, as well as oversee Institute scientific and administrative functions.
Study Implicates Potassium Channel Mutations in Neurodegeneration and Mental Retardation
Sunday, Feb 26, 2006
For the first time, researchers have linked mutations in a gene that regulates how potassium enters cells to a neurodegenerative disease and to another disorder that causes mental retardation and coordination problems. The findings may lead to new ways of treating a broad range of disorders, including Alzheimer's and Parkinson's diseases. The study was funded in part by the National Institutes of Health's National Institute of Neurological Disorders and Stroke (NINDS).
Study Identifies New Mode of Action for Ataxia Gene
Wednesday, Oct 19, 2005
For the first time, researchers have identified how the gene for a hereditary neurodegenerative disease called spinocerebellar ataxia type 1 (SCA1) disables an important group of neurons in the brain. The findings improve understanding of how SCA1 and related diseases develop and may lead to new ways of treating them.
Toxic Interactions from Neighboring Cells May Be Necessary for Huntington’s disease
Tuesday, Sep 27, 2005
A new study suggests that interactions between different cells are critical for the development of Huntington’s disease (HD) and perhaps other neurodegenerative diseases. This study provides the first genetic evidence that cell-cell interactions may be a necessary step in the onset of HD symptoms in a mouse model. This knowledge may lead to new therapeutic strategies to treat HD.
New Members Appointed to National Neurology Advisory Council
Wednesday, Sep 14, 2005
U.S. Department of Health and Human Services Secretary Michael O. Leavitt announces three new appointments and one reappointment to the National Advisory Neurological Disorders and Stroke Council, the major advisory panel of the National Institute of Neurological Disorders and Stroke (NINDS). The NINDS, a component of the National Institutes of Health (NIH), is the nation’s primary supporter of basic, translational, and clinical research on the brain and nervous system. NINDS Director Story Landis, Ph.D., will introduce the new members, who will serve through July 2009, at the Council’s September 15, 2005 meeting.
Silencing Gene Activity Prevents Disease in Model for Huntington's
Tuesday, Jun 7, 2005
Silencing the activity of a mutant gene prevents disease symptoms in a mouse model for Huntington's disease (HD), a new study shows. The study is the first to directly target the underlying problem that causes HD, and it may lead to a new way of treating this disorder.
What's Old is New Again - Antibiotic Protects Nerves By Removing Excess Glutamate
Monday, Feb 7, 2005
A new study shows that a common antibiotic used to treat bacterial infections increases survival rates and delays nerve damage in a mouse model for amyotrophic lateral sclerosis (ALS). The antibiotic works by activating or "turning on" the gene encoding the glutamate transporter in neurons. This finding may lead to new drug treatments for ALS and other neurodegenerative diseases.
Study Using Robotic Microscope Shows How Mutant Huntington's Disease Protein Affects Neurons
Wednesday, Oct 13, 2004
Investigators Explore Selective Silencing of Disease Genes
Using a specially designed robotic microscope to study cultured cells, researchers have found evidence that abnormal protein clumps called inclusion bodies in neurons from people with Huntington's disease (HD) prevent cell death. The finding helps to resolve a longstanding debate about the role of these inclusion bodies in HD and other disorders and may help investigators find effective treatments for these diseases.
Wednesday, Oct 15, 2003
Misbehaving Molecules: 3-Dimensional Pictures of ALS Mutant Proteins Support Two Major Theories About How the Disease is Caused
A new strategy to shut down mutant gene expression in the brain may someday be useful to treat a wide range of hereditary neurodegenerative diseases, such as Huntington’s, Alzheimer’s, and Parkinson’s diseases.
Sunday, May 18, 2003
Dystonia Protein Linked to Problem Common in Other Neurological Disorders
A new study reveals for the first time how gene mutations lead to the inherited form of amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease. The study suggests that the two most prominent theories of how familial ALS (FALS) and other related diseases develop are both right in part.
Monday, Mar 24, 2003
Doubling Up: Researchers Combine a Common Dietary Supplement with an Antibiotic to Treat Lou Gehrig's Disease
A new study links the protein that is impaired in the movement disorder torsion dystonia to a problem that is common to many neurological diseases. The finding may point to new treatments for dystonia, Parkinson's disease, and other disorders.
Friday, Jan 31, 2003
Study Suggests Coenzyme Q10 Slows Functional Decline in Parkinson's Disease
A new study shows that combining the supplement creatine and the antibiotic minocycline significantly slows disease progression and prolongs survival in a mouse model of amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease.
Monday, Oct 14, 2002
Study Finds Psychiatric Disorders are Common in People with Cerebellar Degeneration
Results of the first placebo-controlled, multicenter clinical trial of the compound coenzyme Q10 suggest that it can slow disease progression in patients with early-stage Parkinson's disease (PD). While the results must be confirmed in a larger study, they provide hope that this compound may ultimately provide a new way of treating PD.
Wednesday, Sep 25, 2002
A new study shows that most patients with movement disorders caused by damage to the cerebellum also have psychiatric symptoms. The study suggests that patients with cerebellar diseases may benefit from screening and treatment of psychiatric symptoms.
Minocycline Delays Onset and Slows Progression of ALS in Mice
Thursday, May 2, 2002
Scientists Identify Potential New Treatment for Huntington's Disease
The antibiotic minocycline delays onset and slows progression of symptoms in a mouse model for amyotrophic lateral sclerosis (ALS), a new study shows. The study also revealed that the drug may work by blocking release of a molecule that triggers cell death. The findings may lead to new ways of treating ALS or other neurodegenerative disorders.
Wednesday, Feb 27, 2002
Trial Drugs for Huntington's Disease Inconclusive in Slowing Disease
A drug called cystamine alleviates tremors and prolongs life in mice with the gene mutation for Huntington's disease (HD), a new study shows. The drug appears to work by increasing the activity of proteins that protect nerve cells, or neurons, from degeneration. The study suggests that a similar treatment may one day be useful in humans with HD and related disorders.
Monday, Aug 13, 2001
Scientists Identify Gene for Spinocerebellar Ataxia 2
A large-scale clinical trial that tested the ability of the investigational drugs remacemide and Coenzyme Q10 to slow the progression of Huntington's disease showed that neither drug resulted in any significant improvement for the patients. Although after one year of treatment, the disease seemed to progress more slowly in patients treated with Coenzyme Q10, the investigators say that overall the results are inconclusive as to whether there is real benefit from this drug.
Thursday, Oct 31, 1996
Scientists have identified the gene altered in one of the most common hereditary ataxias, spinocerebellar ataxia 2 (SCA2). The discovery allows improved genetic testing and provides new clues about how genetic mutations cause several neurological disorders, including Huntington's disease. The findings are reported by three different groups in the November issue of Nature Genetics.
New Type of Trinucleotide Mutation Found in Friedreich's Ataxia
Thursday, Mar 7, 1996
Scientists have identified a new type of trinucleotide repeat mutation that leads to Friedreich's ataxia (FA), a rare childhood neurodegenerative disease. The discovery allows accurate screening for carriers of the disease and may lead to the first effective treatments.
Study Links Critical Enzyme to Huntington's, Other Diseases
Thursday, Feb 29, 1996
For the first time, scientists have linked a critical cellular enzyme to the gene defect found in Huntington's and several other hereditary neurological diseases. The finding provides important clues about how these diseases may develop and suggests that a single therapy eventually may be developed to treat them.
A Hereditary Ataxia Caused by Huntington's-Type "Genetic Stutter"
Wednesday, Jun 30, 1993
Scientists have discovered that another nervous system degenerative disorder, spinocerebellar ataxia type 1 (SCA1), has the same type of gene mutation occurring in Huntington's and Kennedy's diseases. In the disease, a normal three-base sequence in the genetic code — cytosine, adenine and guanine, or CAG — is abnormally repeated, according to Drs. Huda Y. Zoghbi, who led one team at the Baylor College of Medicine in Houston, Texas, and Harry T. Orr, who headed the other team at the University of Minnesota in Minneapolis. The same CAG repeat was reported earlier this year in Huntington's disease and in 1991 in the very rare Kennedy's disease, also called X-linked spinobulbar muscular atrophy.
Scientists Isolate "Crown Jewel" — Huntington's Disease Gene
Tuesday, Mar 23, 1993
Scientists have identified the genetic mutation that causes Huntington's disease (HD), a fatal, neurodegenerative disorder characterized by progressive physical and mental deterioration. The discovery, to be reported in the March 26 issue of Cell,* is the culmination of a 10-year-long collaboration between investigators in six laboratory groups around the world with major support from the National Institute of Neurological Disorders and Stroke (NINDS).
NINDS Scientists Isolate Segments Of DNA Sequence That Identify More Than 2,300 Brain Genes
Wednesday, Feb 12, 1992
Using a novel strategy, scientists from the National Institute of Neurological Disorders and Stroke have isolated segments of DNA sequence that uniquely identify more than 2,300 brain genes. The recent data, combined with data from 347 segments sequenced earlier by NINDS scientists, doubles the total number of human genes identified by sequencing, scientists report in the February 13 issue of Nature.
NINDS Scientists Develop Strategy To Speed Gene and Brain Research
Thursday, Jun 20, 1991
Using a novel strategy, scientists at the National Institute of Neurological Disorders and Stroke (NINDS) have isolated key identifying regions of more than 400 genes that work inside the human brain. The scientists say their work should help identify genetic defects that cause brain disease and speed progress of genetics research.