Huntington's Disease News and Research RSS Feed - Huntington's Disease News and Research

Huntington's disease (HD) results from genetically programmed degeneration of brain cells, called neurons, in certain areas of the brain. This degeneration causes uncontrolled movements, loss of intellectual faculties, and emotional disturbance. HD is a familial disease, passed from parent to child through a mutation in the normal gene. Each child of an HD parent has a 50-50 chance of inheriting the HD gene. If a child does not inherit the HD gene, he or she will not develop the disease and cannot pass it to subsequent generations. A person who inherits the HD gene will sooner or later develop the disease. Whether one child inherits the gene has no bearing on whether others will or will not inherit the gene. Some early symptoms of HD are mood swings, depression, irritability or trouble driving, learning new things, remembering a fact, or making a decision. As the disease progresses, concentration on intellectual tasks becomes increasingly difficult and the patient may have difficulty feeding himself or herself and swallowing. The rate of disease progression and the age of onset vary from person to person. A genetic test, coupled with a complete medical history and neurological and laboratory tests, helps physicians diagnose HD. Presymptomic testing is available for individuals who are at risk for carrying the HD gene. In 1 to 3 percent of individuals with HD, no family history of HD can be found.
Modifying huntingtin protein protects against behavioral symptoms in Huntington's model mice

Modifying huntingtin protein protects against behavioral symptoms in Huntington's model mice

There is new hope in the fight against Huntington's disease. Scientists at the Gladstone Institutes discovered that changing a specific part of the huntingtin protein prevented the loss of critical brain cells and protected against behavioral symptoms in a mouse model of the disease. [More]
MGH investigators discover key molecules essential for sensing proteasome dysfunction

MGH investigators discover key molecules essential for sensing proteasome dysfunction

Maintaining appropriate levels of proteins within cells largely relies on a cellular component called the proteasome, which degrades unneeded or defective proteins to recycle the components for the eventual assembly of new proteins. [More]
Novel PET radiotracer reveals epigenetic activity in the human brain for the first time

Novel PET radiotracer reveals epigenetic activity in the human brain for the first time

A novel PET radiotracer developed at the Martinos Center for Biomedical Imaging at Massachusetts General Hospital is able for the first time to reveal epigenetic activity - the process that determines whether or not genes are expressed - within the human brain. [More]
Overcoming gene silencing barriers to target neurological conditions: an interview with Dr Errol de Souza

Overcoming gene silencing barriers to target neurological conditions: an interview with Dr Errol de Souza

Many gene mutations that cause neurological disorders have been identified. For example, in a rare neurological disorder such as Huntington’s disease, an autosomal dominant mutation through expansion of CAG (cytosine-adenine-guanine) triplet repeats in the gene coding for the Huntingtin protein results in abnormal protein production. [More]
Researchers discover pathway linking oxidative stress and cysteine in Huntington's disease

Researchers discover pathway linking oxidative stress and cysteine in Huntington's disease

Researchers at Johns Hopkins Medicine report they have identified a biochemical pathway linking oxidative stress and the amino acid cysteine in Huntington's disease. [More]
Huntington's disease monkey model embodies full array of symptoms similar to human patients

Huntington's disease monkey model embodies full array of symptoms similar to human patients

Transgenic Huntington's disease monkeys display a full spectrum of symptoms resembling the human disease, ranging from motor problems and neurodegeneration to emotional dysregulation and immune system changes, scientists at Yerkes National Primate Research Center, Emory University report. [More]
Researchers develop new screening system to measure protein aggregates

Researchers develop new screening system to measure protein aggregates

Researchers from the Massachusetts Institute of Technology and Boston University have developed a system capable of quickly screening millions of yeast cells to measure protein aggregates. [More]
Deutetrabenazine may help improve chorea symptoms in patients with HD

Deutetrabenazine may help improve chorea symptoms in patients with HD

People with Huntington disease (HD) experienced improvements in chorea while taking deutetrabenazine (SD-809) compared to placebo, according to a paper published today in the Journal of the American Medical Association. [More]
Deuteration offers alternative chorea treatment for Huntington disease patients

Deuteration offers alternative chorea treatment for Huntington disease patients

Treatment with a deuterated form of tetrabenazine has resulted in improved motor signs among patients with Huntington disease, making it a potential treatment for chorea, trial findings show. [More]
Neurofibromatosis causes benign tumor formation in normal nervous tissue

Neurofibromatosis causes benign tumor formation in normal nervous tissue

Although neurofibromatosis (NF) is not commonly discussed, it affects more than 2 million people worldwide. [More]
Study shows many people may have potential to develop Huntington's disease

Study shows many people may have potential to develop Huntington's disease

More people may have the potential to develop Huntington's disease than previously thought, according to a study published in the June 22, 2016, online issue of Neurology, the medical journal of the American Academy of Neurology. [More]
TSRI scientists develop novel technique for finding drug candidates

TSRI scientists develop novel technique for finding drug candidates

Scientists at The Scripps Research Institute have developed a powerful new method for finding drug candidates that bind to specific proteins. [More]
Mice implanted with human glia cells exhibit reduced symptoms of Huntington's disease

Mice implanted with human glia cells exhibit reduced symptoms of Huntington's disease

Researchers have successfully reduced the symptoms and slowed the progression of Huntington's disease in mice using healthy human brain cells. The findings, which were published today in the journal Nature Communications, could ultimately point to a new method to treat the disease. [More]
New research highlights value of exercise for humans looking to keep their brains fit

New research highlights value of exercise for humans looking to keep their brains fit

The relentless desire by mice to run on cage "exercise" wheels has helped explain at a molecular level how exercise benefits a mammal's brain. [More]
New research in mice provides better understanding of how HD begins and develops

New research in mice provides better understanding of how HD begins and develops

Mice transplanted with cells grown from a patient suffering from Huntington's disease (HD) develop the clinical features and brain pathology of that patient, suggests a study published in the latest issue of Acta Neuropathologica by CHA University in Korea, in collaboration with researchers at Université Laval in Québec City, Canada. [More]
Exercise may help reduce toxicity caused by glutamate build-up in the brain

Exercise may help reduce toxicity caused by glutamate build-up in the brain

In a new study published today in Applied Physiology, Nutrition, and Metabolism, scientists from the University of Guelph have found that exercise has the potential to decrease toxic build-up in the brain, reducing the severity of brain disorders such as Huntington's disease. [More]
Unlocking the dark proteome: an interview with Dr Kriwacki

Unlocking the dark proteome: an interview with Dr Kriwacki

The term dark proteome refers to proteins whose structural features and thus functions are not well understood. Many proteins within the dark proteome do not fold into stable three-dimensional structures. These proteins are called intrinsically disordered proteins (IDPs) and feature highly flexible, disordered confirmations. [More]
New way of understanding neurodegeneration may lead to novel treatments

New way of understanding neurodegeneration may lead to novel treatments

University of Adelaide researchers have developed a new theory for the causes of dementia and other neurodegenerative diseases, involving an out-of-control immune system. [More]
Key gene changes reveal four previously unknown conditions within schizophrenia

Key gene changes reveal four previously unknown conditions within schizophrenia

Changes in key genes clearly define four previously unknown conditions within the umbrella diagnosis of schizophrenia, according to a study led by researchers from NYU Langone Medical Center published online April 28 in EBioMedicine, a Lancet journal. Cases associated with changes in each of the four genes were different from each other in terms of symptoms, intelligence level and other disease features. [More]
Innovative strategy can reverse symptoms in neurodegenerative diseases

Innovative strategy can reverse symptoms in neurodegenerative diseases

Alzheimer's and Parkinson's disease are the two most common neurodegenerative disorders worldwide and cause untold suffering to millions of patients and their families. Treatments for these diseases are limited, and no cures exist. Now, a new study describes an innovative strategy that reverses symptoms in these neurodegenerative diseases - at least in fruit flies which had been genetically altered to model the diseases. [More]
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