Researchers Develop First Transgenic Monkey Model of Huntington’s DiseaseFriday, 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 AtaxiaWednesday, 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 DisordersThursday, 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 PunchTuesday, 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 DirectorWednesday, 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 RetardationSunday, 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 GeneWednesday, 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.
New Members Appointed to National Neurology Advisory CouncilWednesday, 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'sTuesday, 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 GlutamateMonday, 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 NeuronsWednesday, Oct 13, 2004
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.
Fact Sheet Misbehaving Molecules: 3-Dimensional Pictures of ALS Mutant Proteins Support Two Major Theories About How the Disease is
CausedSunday, May 18, 2003
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.
Fact Sheet Doubling Up: Researchers Combine a Common Dietary Supplement with an Antibiotic to Treat Lou Gehrig's DiseaseFriday, Jan 31, 2003
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.
Fact Sheet Study Suggests Coenzyme Q10 Slows Functional Decline in Parkinson's DiseaseMonday, Oct 14, 2002
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.
Fact Sheet Study Finds Psychiatric Disorders are Common in People with Cerebellar DegenerationWednesday, 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 MiceThursday, May 2, 2002
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.
Fact Sheet Scientists Identify Potential New Treatment for Huntington's DiseaseWednesday, Feb 27, 2002
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.
Fact Sheet Trial Drugs for Huntington's Disease Inconclusive in Slowing DiseaseMonday, Aug 13, 2001
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.
Fact Sheet Scientists Identify Gene for Spinocerebellar Ataxia 2Thursday, 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 AtaxiaThursday, 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 DiseasesThursday, 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 GeneTuesday, 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 GenesWednesday, 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 ResearchThursday, 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.