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.

Research has shown that cells have a cleanup system for handling protein "stress," and some studies suggest the possibility of developing therapeutic drugs that would work by giving the system a boost. But a new study published in Neuron suggests that during prolonged stress, the cleanup system can suppress vital cell functions or even actively kill the cell.

Increasing the activity of a key protein in the bloodstream slows the buildup of a toxic substance in the brains of mice with the gene mutation for Alzheimer's disease (AD). It also prevents some memory problems, a new study shows. If the approach works in humans, it may eventually lead to a way of preventing or halting AD.

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.

Although seizures are not a common symptom of Alzheimer's disease (AD), the brains of people with AD could be humming with seizure-like activity, interrupted by quiet rebound periods that do more harm than good.

In preliminary results, researchers have shown that a drug which mimics the effects of the nerve-signaling chemical dopamine causes new neurons to develop in the part of the brain where cells are lost in Parkinson's disease (PD). The drug also led to long-lasting recovery of function in an animal model of PD. The findings may lead to new ways of treating PD and other neurodegenerative diseases.

For the first time, researchers have identified a specific form of amyloid beta protein that causes memory impairment long before amyloid plaques and neurodegeneration appear in a mouse model of Alzheimer's disease (AD). The finding may lead to new ways of diagnosing and possibly even preventing the disease.

A new study in mice suggests that Alzheimer's disease (AD) may be triggered when adult neurons try to divide. The finding helps researchers understand what goes wrong in the disease and may lead to new ways of treating it.

For decades, researchers have tried to understand what triggers clusters of neurons to begin signaling excessively in epilepsy. A new study shows that, in many cases, the answer resides in star-shaped support cells called astrocytes. The finding may lead to new ways of treating epilepsy.

Most people with a rare type of dementia called primary progressive aphasia (PPA) have a specific combination of prion gene variants, a new study shows. The study is the first to link the prion protein gene to this disorder. The researchers also looked at the prion protein gene in people with Alzheimer's disease and amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease) and did not find any association with specific gene variants in those disorders.

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.

While several treatments are currently available for Alzheimer's disease (AD), none of them can slow or halt the course of this devastating disorder. In a new study, researchers have now identified three compounds that inhibit an enzyme believed to be involved in the process that leads to AD. This discovery may lead to new treatments that can stop the disease process in its tracks.

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.

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.
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For the first time, researchers have shown that an experimental vaccine can reduce the amount of neurodegeneration in a mouse model for Parkinson's disease. The finding suggests that a similar therapy might eventually be able to slow the devastating course of Parkinson's disease in humans.
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Scientists who developed the first yeast model of Parkinson's disease (PD) have been able to describe the mechanisms of an important gene's role in the disease. Tiago Fleming Outeiro, Ph.D., and Susan Lindquist, Ph.D., of the Whitehead Institute for Biomedical Research in Cambridge, Massachusetts, studied the gene's actions under normal conditions and under abnormal conditions to learn how and when the gene's product, alpha-synuclein, becomes harmful to surrounding cells. The scientists created a yeast model that expresses the alpha-synuclein gene, which has been implicated in PD. Yeast models are often used in the study of genetic diseases because they offer researchers a simple system that allows them to clarify how genes work.
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Scientists investigating a rare familial form of early-onset Parkinson's disease have discovered that too much of a normal form of the alpha-synuclein gene may cause Parkinson's disease. The finding, reported in the October 31, 2003, issue of Science, shows that abnormal multiplication of the alpha-synuclein gene can cause the disease.
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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.
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A new study has begun to unravel the mysteries of protein interactions that govern the strength of nerve cell connections, or synapses, in the brain. The findings give researchers a better understanding of how synapses function during learning and memory, and they may lead to new insights about such neurological disorders as Parkinson's and Alzheimer's diseases.

Recent studies provide strong evidence that genetic factors influence susceptibility to the common, late-onset form of Parkinson's disease (PD). The findings improve scientists' understanding of how PD develops and may lead to new treatments or even ways of preventing the disease.
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The first meeting of the NINDS Parkinson's Disease Implementation Committee (PDIC) was held July 31, 2000 at the National Institutes of Health, Neuroscience Center in Rockville, Maryland. The Committee identified several areas of Parkinson's disease research that will receive the highest priority in the coming weeks, including clinical trials and gene research.

The National Institute of Neurological Disorders and Stroke (NINDS), in collaboration with the National Center for Research Resources (NCRR) and the Office for Research on Minority Health (ORMH), recently awarded grants to five minority institutions under a new funding mechanism called Specialized Neuroscience Research Programs at Minority Institutions (SNRP).

For years, researchers have probed the mysteries of neural stem cells -- immature cells that can differentiate into all the cell types that make up the brain -- with the idea that they might be useful for treating brain disorders such as Parkinson's disease. Important new animal research now suggests that these cells may be effective in treating a much broader array of brain diseases than previously anticipated, including Alzheimer's disease and many childhood brain disorders.

Stanley B. Prusiner, M.D., a long-time grantee of the National Institutes of Health (NIH), is the recipient of the 1997 Nobel Prize in physiology or medicine for his discovery of an unusual class of infectious particles called prions. Prions are believed to be responsible for a group of diseases that include "mad cow" disease. Prusiner, who is professor of neurology, virology, and biochemistry at the University of California, San Francisco (UCSF), has received more than 56 million dollars in research grant support from NIH during the last three decades.

A protein widely distributed in tissues throughout the body, with the highest concentration in the brain, has been shown to be a specific marker in the spinal fluid of humans and animals infected with transmissible spongiform encephalopathies, scientists say. This discovery paves the way for the development of an improved test for the diagnosis of Creutzfeldt-Jakob disease in humans and encephalopathies in animals. The test could enable precise identification of disease in British cattle presently targeted for slaughter because of suspected infection with bovine spongiform encephalopathy, known as Mad Cow disease.

Scientists at the National Institute of Neurological Disorders and Stroke (NINDS) have discovered that adding a substance called beta amyloid to normal skin cells causes the cells to exhibit the same type of molecular dysfunction previously demonstrated in skin cells of patients with Alzheimer's disease (AD). This step may lead to a new explanation of memory loss, one of the earliest and most common symptoms of the disease.

Scientists at the National Institute of Neurological Disorders and Stroke (NINDS) in Bethesda, MD, and the Burke Medical Research Institute at Cornell Medical College in White Plains, NY, have discovered physiological differences in the skin cells of those with Alzheimer's disease (AD), a finding that could lead to a standard battery of skin tests for diagnosing the disease.

Officials at the National Institute of Neurological Disorders and Stroke (NINDS) hailed the identification of a gene associated with the familial form of ALS (Lou Gehrig's disease). "This discovery is extremely important because it marks the first identification of a specific gene for a neurodegenerative disease of adult life," said Carl M. Leventhal, M.D., director of the NINDS program that contributed to support for the research reported in the March 3 issue of Nature*. "It also suggests a likely mechanism for the damage to nerve cells in familial ALS and, possibly, other brain disorders."

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.

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.

Scientists at the National Institute of Neurological Disorders and Stroke (NINDS) have linked three outbreaks of Creutzfeldt-Jakob disease (CJD) in Europe and Israel to a genetic mutation found in the outbreaks' victims.