Articles tagged with Huntingtons Disease
Researchers found that a gaseous molecule called nitric oxide can trigger brain cell death in neurodegenerative diseases. The study discovered a new molecular pathway that leads to cellular suicide, offering new potential for diagnosis and treatment.
A study reveals that an enzyme involved in multiple disorders also generates toxic protein fragments in Huntington's disease, causing neuron death. Researchers propose inhibiting MMP family members as a potential therapeutic strategy.
Researchers identified how human mutant 'huntingtin' proteins form into large clumps, killing brain cells and leading to progressive Huntington's disease. The discovery reveals that these clusters place a steady stress on cells over time, providing potential targets for targeted treatments.
Researchers linked kynurenic acid to cognition and found that reducing its levels in mice improved their cognitive function. The study suggests a promising strategy for enhancing memory and treating brain diseases such as Alzheimer's and schizophrenia.
A new estimate suggests that Huntington's disease affects at least 12.4 per 100,000 people in England and Wales, likely an underestimate due to stigma and lack of genetic testing. The true prevalence may be higher, with implications for healthcare services and research into treatments.
The collaboration aims to identify genes and novel drug targets related to the onset and progression of Huntington's disease. The team will use induced pluripotent stem cells from patients with HD to screen for drugs that might delay, prevent, or reverse the devastating condition.
Researchers discovered a small group of genes in fruit flies that likely play important roles in regulating the formation of plaque-like protein aggregates within cells. The identified genes have human counterparts and may be manipulated to stop or slow the formation of these aggregates, which are a hallmark of Huntington's disease.
Researchers have discovered Ku70 to be a vital component in the DNA repair process for neurons, crucial in preventing polyQ diseases like Huntington's. Boosting Ku70 levels rescues mutant huntingtin-induced neurodegeneration in mouse models of HD.
A study by Einstein researchers suggests that a mutated protein accumulation may be key to understanding the disease. The defective huntingtin protein interferes with cellular autophagy, leading to toxicity and cell death.
UC Irvine researchers have sequenced the Hydra genome, discovering genes linked to Huntington's disease and beta-amyloid plaque formation in Alzheimer's. The genome sequencing advances research on regeneration, stem cells, and patterning, offering potential new treatments for various injuries and diseases.
Results from an open-label extension study demonstrate that subjects treated with Xenazine experienced a statistically significant reduction in chorea score after 80 weeks of treatment. The primary efficacy endpoint showed a mean reduction in Total Maximal Chorea (TMC) score of 4.6 UHDRS units.
Researchers at the University of Western Ontario have identified a protective pathway in the brain that may help explain why symptoms of Huntington's disease appear later in life. This finding could lead to new treatments for the devastating genetic disorder, which is caused by cell death in specific brain regions.
A clinical trial of the experimental drug dimebon found it to be safe and improved cognition in patients with Huntington's disease. The study, led by University of Rochester Medical Center neurologist Karl Kieburtz, showed statistically significant benefits for patients taking the drug compared to those receiving a placebo.
A medication called latrepirdine has been found to be well-tolerated in patients with mild to moderate Huntington's disease. The treatment showed improved average scores on an evaluation measuring overall cognitive function, suggesting potential benefits for cognition.
A toxic form of the neural protein Elk-1 is linked to three degenerative brain disorders: Parkinson's disease, Alzheimer's disease, and Huntington's disease. The study found that this modified form of Elk-1 strongly associates with pathological markers present in diseased tissue from these diseases.
A new study has uncovered a key cellular mechanism that alters brain cell function in Huntington's disease and identified a possible treatment for the disease. Researchers found that excessive NMDA receptors outside synapses lead to reduced brain cell survival signals and disruption in brain function.
Scientists at Duke University Medical Center have identified compounds that activate a master regulator to increase the supply of protein chaperone molecules, which help fold proteins properly. This discovery provides a new approach to address protein misfolding, a common factor in degenerative nerve diseases.
Two studies found that small changes to a protein's chemistry can eliminate signs of Huntington's disease in mice. Researchers identified two amino acids critical for regulating the toxic protein, suggesting potential targets for drug therapy.
Researchers discovered a molecular switch that prevents Huntington's disease from developing in mice, providing new hope for treating the genetic disorder. The study suggests that phosphorylation of specific amino acids near the huntingtin protein can prevent the onset of symptoms.
The kinase IKK phosphorylates mutated Huntingtin protein to promote its removal, but also increases neurotoxicity in later stages of the disease. This dual role highlights the complexity of IKK's function in Huntington's disease.
Researchers found that normal synaptic activity protects the brain from misfolded proteins associated with Huntington's disease, while excessive extrasynaptic activity enhances their deadly effects. Low doses of Memantine successfully treated Huntington's disease in a mouse model by preserving normal synaptic electrical activity.
Researchers at Caltech have shown that a highly specific intrabody can stall the development of Huntington's disease in various mouse models. The treatment successfully attenuated symptoms and increased life span by targeting an amino-acid sequence unique to the mutant huntingtin protein.
The Gladstone Institute of Neurological Disease and partners will use induced pluripotent stem (iPS) cell technology to develop human neurons with Huntington's disease characteristics, offering hope for new treatments. The goal is to understand the molecular differences between mice and humans that lead to ineffective therapies.
Dr. Roger Rosenberg has been awarded the first Medal for Scientific Achievement by the World Federation of Neurology for his contributions to Alzheimer's disease research. The award recognizes his work on molecular genetics and a vaccine against the disease.
A recent study found that transplanted neurons develop disease-like pathology in Huntington's patients, raising concerns about the therapeutic potential of cell transplantation therapy. The research suggests new mechanisms involved in the development of the disease and offers a new direction for developing novel therapeutic strategies.
A novel DNA repair pathway, referred to as DNA hairpin repair (HPR), targets TNR hairpin removal in the daughter strand to ensure fidelity of TNR sequences. This finding may be responsible for TNR instability in diseases such as Huntington's disease.
The study reveals that the mutated huntingtin gene activates JNK3 enzyme, inhibiting axonal transport and leading to neuronal cell death. The mechanism explains the late onset of the disease, as young neurons have a robust transport system that gradually declines with age.
Researchers estimated the probability of male carriers having a child with Huntington's disease, finding it ranges from 1/6,241 to 1/951. This estimate provides a baseline for genetic counselors and high normal allele carriers for family planning.
Researchers at Johns Hopkins have discovered a tiny protein called Rhes responsible for brain cell damage in Huntington's disease. The findings explain the unique pattern of brain damage and offer a strategy for new therapy.
Researchers at the Wellcome Trust Sanger Institute have identified a set of brain proteins responsible for various neurological disorders. These proteins are found to be defective in molecular machines that control communication between nerve cells and learning processes.
Researchers created short lengths of molecules that resemble ribonucleic acid to bind to CAG repeats, preventing cells from creating abnormal proteins. These compounds were effective against Huntington's and Machado-Joseph diseases, but further tweaking is needed to minimize effects on normal proteins.
Researchers from USC have discovered a potential treatment for Huntington's disease using gene therapy. They found that over-expressing the RCAN1-1L gene can rescue cells from the toxic effects of the disease. This breakthrough offers new avenues for treatment and may have implications for other CAG repeat-related diseases.
Researchers used mouse models to study Huntington Disease, finding significant protein alterations as early as 2 weeks before symptoms appear. These changes may affect late-stage disease by altering biochemical activity in the brain.
Researchers have designed tiny RNA molecules that can reduce production of the damaging Huntingtin protein in nearly half of people with the disease. An additional set of four small interfering RNAs may benefit an additional 25 percent of patients.
The new center aims to prevent, treat, or cure Huntington's disease by 2020 through cutting-edge research and collaboration with pharmaceutical companies. Investigators will focus on identifying potential drug targets and developing innovative technologies to modulate the disease.
Researchers at the University of Pittsburgh School of Medicine discovered a molecular '2-step' process that may lead to protein clumping in Huntington's disease. The study found that a slight lengthening of the polyglutamine sequence disrupts neighboring regions, initiating aggregation behavior. This discovery could provide new targets...
Researchers have discovered a plant model that mimics human DNA patterns, allowing for the study of genetic diseases such as Huntington's and Fragile X syndrome over multiple generations. This breakthrough could pave the way for better understanding and potential treatments for these debilitating conditions.
UT Southwestern researchers have discovered a connection between disrupted calcium metabolism in nerve cells and a fatal genetic neurological disorder called spinocerebellar ataxia 3. The study suggests that blocking excessive calcium release may alleviate symptoms, with results showing improved coordination and slowed brain atrophy in...
Researchers have identified compounds that block the activity of a specific enzyme, preventing brain injury and improving survival in fruit flies with Huntington's disease. The findings could lead to better treatments for degenerative diseases such as Alzheimer's and Parkinson's.
Studies found that patients with Huntington's disease have higher levels of immune-system signaling molecules, called cytokines, in their brain tissue. This suggests that the protein produced by the Huntington's disease genetic mutation is causing an overactive immune response, leading to damage to neurons in the brain.
Researchers found abnormally high levels of cytokines in blood of Huntington's disease gene carriers years before symptoms appeared. White blood cells and microglia also showed hyperactivity, suggesting abnormal immune activation could be an early disease hallmark.
A recent study found high levels of IL-6 in affected individuals over a decade before nervous system symptoms began to manifest. This discovery challenges current understanding of the physiological basis of Huntington's disease and may lead to new early intervention strategies.
Research using Magnetic Resonance Imaging technology has confirmed Huntington's disease before symptoms appear, allowing for early treatment. The study identified extensive white matter degeneration, a hallmark of the disease, which can help explain its complex motor and cognitive problems.
Researchers at Emory University developed an intrabody that binds to mutant huntingtin, reducing clumps and alleviating motor problems in mice. The study suggests a strategy for dissecting harmful effects of protein aggregates in other neurodegenerative diseases.
Researchers found that faulty RNA plays a key role in the onset and progression of neurodegenerative diseases. They discovered that altering the RNA structure can mitigate toxicity, suggesting a common component between different types of human triplet repeat expansion diseases.
Researchers have created a genetically altered monkey model that replicates symptoms of Huntington's disease, allowing for a deeper understanding of the disease mechanisms. This breakthrough could lead to major advances in developing new treatments for neurological diseases.
Research published in BMC Neuroscience found that physical activity from juvenile age delays the onset of specific motor deficits in a mouse model of Huntington's disease. The study suggests that benefits stem from stimulation of neuronal receptors and other molecules that prolong normal function and delay motor deficits.
Researchers have identified promising new drug targets for Huntington's disease, which can stimulate autophagy and alleviate the toxicity of malformed proteins. Candidate drugs include verapamil and clonidine, which have been shown to be safe and effective in cell-based models.
A new stem cell technique has been developed by UC Irvine researchers, which blends two existing methods to improve cell survival rates and increase the efficiency of inserting DNA into cells. This approach is up to 100 times more efficient than current methods at producing human embryonic stem cells with desired genetic alterations.
Researchers identified a region on HIP1 that could bind HIPPI, potentially leading to the degeneration of nerve cells. By targeting this interaction, they hope to design a drug that can prevent the disease.
A recent study published in Neuron found that the damaged protein involved in Huntington's disease causes problems at the synapse early in its development, rather than after it is cut and imported into the nucleus. This discovery may lead to new targets for potential drug therapies targeting genes involved in synaptic transmission.
Researchers have discovered a new mechanism by which abnormal protein in Huntington's disease causes neurodegeneration. Suppressing abnormally high neurotransmission and calcium channel activity may delay onset and progression of the disease.
Scientists at the Weizmann Institute have proposed a mechanism that explains the precision of trinucleotide repeat diseases like Huntington's. They suggest that the genes carrying the disease code accumulate more DNA repeats over time until a critical threshold is crossed.
Scientists have discovered a new approach to treat Huntington's disease using stem-cell therapy, which created thousands of new medium spiny neurons in mice. The treatment resulted in improved health and lifespan for the treated mice.
A new study by Tufts University biologists suggests that people with Huntington's disease have better health earlier in life, leading to increased offspring production. The researchers propose that elevated levels of tumor suppressor protein p53 may contribute to these health benefits and improved immune function.
Researchers at the University of Leeds have discovered a naturally occurring protein preventing 57 genes from operating normally in Huntington's sufferers' brains. Cancer drugs targeting this protein could halt its destructive nature.
Researchers at McMaster University have discovered a new molecular zip code and potential drug target for Huntington's disease. They found that mutant huntingtin protein can be prevented from entering the nucleus by kinase inhibitors, which may lead to effective treatment options.
Researchers found a marked resemblance between molecular etiology of neurons in animal models and humans with HD, making them relevant for studying the disease and testing treatments. The study's findings have important consequences for preclinical drug testing.
Researchers at UT Southwestern Medical Center have found that a drug called tetrabenazine (TBZ) prevents death of brain cells in mice genetically engineered to mimic Huntington's disease. The study sheds light on the biochemical mechanisms involved in the disease and suggests new avenues of study for preventing brain-cell death.
Researchers found that daily treatments of Alprazolam and chloral hydrate improved learning, arousal, and regular sleep patterns in HD mice. The study suggests that restoring normal sleep-wake activity could slow cognitive decline, improving quality of life for patients.