Articles tagged with Huntingtons Disease
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.
Researchers found that a breakdown of myelin in the developing brain may contribute to the progression of Huntington's disease. The study, led by Dr. George Bartzokis at UCLA, suggests that an abnormality in the Htt gene affects myelin nourishment, leading to neuron death and disease symptoms.
Researchers found that individuals with minimal motor problems at the beginning of the study were nearly five times more likely to be diagnosed with Huntington's disease a year and a half later. Those who performed worse on cognitive tests, such as psychomotor speed, were also at increased risk.
Researchers have identified more than 200 new proteins that bind to normal and mutant forms of the protein causing Huntington’s disease. The study suggests these proteins may be potential drug targets for treating the incurable disease, which affects 30,000 Americans annually.
Researchers at Baylor College of Medicine have identified more than 200 new proteins that interact with the mutated protein causing Huntington's disease, offering potential therapeutic targets. These interactions may modulate the effects of the protein, either improving or worsening symptoms, and could help accelerate disease onset.
Researchers have found a way to induce autophagy, a process where cells recycle waste material, including misfolded proteins. By administering small molecules that enhance this process, they aim to stall the onset of Huntington's-like symptoms in humans.
Researchers have discovered that faulty DNA repair contributes to the onset of Huntington's disease. The study suggests that targeting a key enzyme in oxidative lesion repair may offer a way to slow or stop the disease.
Researchers found that a miscue in the body's genetic repair system may cause Huntington's disease, a fatal condition that destroys the nervous system. The study revealed that repeated tracts of replacement repair segments become toxic and accelerate cell death.
Researchers discover that enhancing proteasome function can recover UPS function and improve cell viability in HD model and patient cells. This breakthrough suggests a potential therapeutic approach to address the underlying protein misfolding disorder of HD.
Associate professor Ray Truant's lab has discovered molecular 'zip codes' in the huntingtin protein that dictate its location within brain cells. The research aims to redirect the mutant protein's accumulation and develop new drugs to treat Huntington's Disease.
Researchers at Mayo Clinic have discovered a protein interaction that may explain how Huntington's disease affects the brain, leading to dramatic accumulation of cholesterol.
Researchers at UT Southwestern Medical Center have discovered that memantine and riluzole are the most effective compounds in keeping cells alive under conditions mimicking Huntington's disease. The study provides a systematic comparison of various glutamate pathway inhibitors, indicating memantine holds the most promise for HD treatment.
Research reveals a metabolic defect underlying Huntington's disease, causing temperature dysregulation in brain regions like the striatum. The findings may explain symptoms like weight loss and could lead to new therapeutic avenues.
Researchers at Cambridge University have developed an effective new method to test cognitive decline in mice with Huntington's disease using an automated touch screen. The tool allows for minimal movement by the mouse and is less stressful, making it a valuable asset in studying neurological disorders.
A unique medical research study has begun evaluating 1,001 individuals at risk of developing Huntington's disease who do not know whether they carry the genetic defect. The PHAROS study aims to identify early signs of the disease and inform clinicians in designing better studies for new drugs.
Researchers identified minocycline's role in blocking poly(ADP-ribose) polymerase-1 (PARP-1), a protein linked to inflammation and cell death. The study suggests minocycline's potential as a treatment for neurodegenerative diseases, but raises concerns about its effects on cancer risk and gender differences.
Researchers at the University of British Columbia have successfully prevented Huntington disease in a mouse model by blocking the cleavage of the mutant huntingtin protein. This discovery provides new hope for effective treatment and potentially a cure for the devastating disorder.
Researchers used gene therapy to deliver glial-derived neurotrophic factor (GDNF) directly to the brain cells of mice with Huntington's disease, protecting neurons from degeneration. The study showed improved behavioral function and reduced symptoms in mice treated with GDNF, suggesting a new approach to forestall disease progression.
A study published in Nature Neuroscience reveals that polyQ-AR, a mutated protein in Kennedy disease, inhibits fast axonal transport by activating JNK enzyme. This inhibition leads to selective neuron death and loss of motor neurons.
Scientists at Emory University Health Sciences Center have identified the protein HAP1 as crucial for neuronal function and trafficking. The discovery may lead to new treatments for Huntington's disease by understanding how mutant huntingtin affects cellular transport. Research has implications for other neurodegenerative disorders.
Researchers found that cystamine and a related drug increase levels of protective protein HSJ1b, which helps neurons survive in Huntington disease. This may lead to potential treatments for the neurodegenerative disorder.
Researchers at MIT have discovered a compound called B2 that promotes the formation of large protein inclusions, which may help stop Huntington's disease progression. The compound also shows promise for treating Parkinson's disease, another neurodegenerative disorder caused by misfolded proteins.
Neural transplants have provided long-term clinical benefits to three patients with Huntington's disease, improving motor and cognitive function. The procedure also led to focal improvements in brain metabolic activity, while secondary clinical alterations were observed due to the ongoing disease process.
A recent study found that tetrabenazine, a medication currently available in Europe and Canada, showed significant improvement in reducing chorea, a hallmark symptom of Huntington's disease. The study involved 84 patients and was led by Dr. Kathleen M. Shannon.
Researchers used PET and MR imaging to show white matter volume loss precedes clinical symptoms of Huntington's disease. The study suggests a new approach to preventing the disease at an early stage by monitoring brain tissue volumes and basal ganglia dysfunction.
Tetrabenazine has been shown to decrease involuntary movement by 25% and improve patient outcomes. The medication targets the excessive movements caused by the disease's mutant protein, providing relief for patients with tasks such as eating and walking.
Researchers found that polyglutamine proteins can destabilize the cell's system by interfering with other proteins having difficulty folding, leading to massive consequences. The study suggests a common mechanism may underlie various neurodegenerative diseases, including Huntington's and ALS.
A study published by University of Iowa researchers has identified the CHIP protein as a crucial component in managing neurodegenerative diseases like Huntington's and Alzheimer's. By suppressing misfolded proteins, CHIP may provide a promising route to therapy for these devastating brain disorders.
The Mayo team identified a key protein that fails to recognize specific forms of DNA under certain conditions, leading to defective DNA repair. This discovery holds promise for designing new therapies for Huntington's disease and other neurodegenerative disorders.
Researchers found that abnormally long glutamine tracts in proteins can cause nerve cells to deteriorate and die. The study suggests that understanding the molecular mechanism behind polyglutamine diseases may lead to the development of new treatments, including small molecule drugs.
Researchers have found that Clioquinol, an old antibiotic, may interrupt the production of mutant huntingtin protein in neurons, reducing its toxic effects and potentially slowing down Huntington's disease progression. The study suggests a new potential treatment for the degenerative brain disorder.
Researchers discovered that prions can rapidly 'remodel' good protein into bad, shedding important light on the molecular machinery behind infectious brain diseases. This process may also help explain the progression of Alzheimer's, Parkinson's and Huntington's diseases.
Researchers at Yerkes develop a transgenic nonhuman primate model of Huntington's disease to study disease development and develop treatments and prevention options. The model will be used in high-resolution imaging scans and behavioral studies.
Scientists have discovered a way to treat Huntington's disease by targeting multiple proteins and genetic pathways simultaneously. This breakthrough has the potential to provide new hope for patients with this devastating neurodegenerative disorder.
A study published in Neuron found that the abnormal HD protein selectively binds to and increases the level of p53 in cells, leading to increased cell death and mitochondrial dysfunction. This overactivation also causes behavioral abnormalities in mice engineered to have HD.
Researchers at UCLA Neuropsychiatric Institute developed a mouse model showing that mutant HD proteins exert influence on nearby brain cells, which interact with target cells to spark disease. The study provides direct genetic evidence for the role of cellular interactions in Huntington's disease progression.
The Rush University Medical Center has been designated as the first HDSA Center of Excellence in Illinois, providing a range of medical and social services to Huntington's disease patients. The center will also explore the creation of a regional network of care providers, including end-of-life care.
Steve Finkbeiner, a Gladstone investigator, has won the prestigious Lieberman Award for his groundbreaking research on Huntington's disease. The award includes $150,000 in funding to build on his findings using a custom-designed robotic microscope that tracks changes in cells over long periods.
Researchers have identified the KMO enzyme as a potential therapeutic target for Huntington's disease, with a chemical compound already available to inhibit its activity. The discovery could take research in a new direction towards microglial cells, which are thought to play an important role in the progression of the disease.
Researchers at U Iowa have made significant breakthroughs in treating Huntington's disease by reducing protein levels in genetically engineered mice. The study, published in PNAS, demonstrates the effectiveness of RNA interference in improving HD-like symptoms in a mouse model.
Scientists find that a region near the head of the ataxin-3 protein counterbalances toxicity caused by excessive polyglutamine repeats, potentially offering a therapeutic solution for Machado-Joseph disease and related disorders. The study suggests that removing or altering this region can accelerate disease progression.
A UCI study found that combinatorial drug therapies halted brain-cell damage in fruit flies with mutated Huntingtin protein, showing potential for treating neurodegenerative diseases. The treatment combines compounds targeting different cellular processes with no toxic side effects.
Researchers developed a model linking Huntington's disease mutation to cell death, revealing calcium signaling as a key defect. A new drug, enoxaparin, prevented inappropriate calcium release and cell death in mouse neurons carrying the mutant huntingtin gene.
Researchers identified HIP14 as a key enzyme in palmitoylation, a process essential for normal nervous system function. The discovery sheds light on mechanisms underlying diseases like Alzheimer's and Huntington Disease.
The University of South Florida has been designated a Center of Excellence by the Huntington's Disease Society of America, offering comprehensive medical and social services to patients and their families. The center will also focus on research, including studies on stem cells and potential new therapies for the disease.
A Northwestern University team discovered that mutant Huntingtin protein aggregates bind to the proteasome machine, preventing complete degradation of proteins and leading to disease. This interference causes a cumulative negative effect, resulting in the buildup of damaged proteins.
The study found that inclusion bodies in Huntington's disease are beneficial coping responses, sequestering mutant huntingtin protein and prolonging neuron survival. The researchers used robotic microscopy to track changes in individual neurons over time, enabling them to identify factors that predict cell fate.
Researchers found minor molecular abnormalities in Huntington's disease cells, but only specific groups degenerate and die. The study suggests that therapies for neurodegenerative diseases like Alzheimer's and Parkinson's may need to address multiple cellular processes.
A study by Tufts University researcher Catherine Freudenreich reveals that cells with certain DNA mutations may activate a surveillance system to repair damaged DNA, leading to cell death. The findings could lead to advances in treating diseases such as Huntington's disease and muscular dystrophy.
Scientists used gene therapy to deliver RNA that silenced the disease-causing SCA1 gene in mice with spinocerebellar ataxia 1, preventing neurodegeneration. The approach also protected brain cells from destruction and prevented protein clump buildup.
A new mouse model of SBMA has been created, showing that the abnormal androgen receptor interferes with vascular endothelial growth factor (VEGF) production, leading to motor neuron degeneration. The study suggests VEGF may play a pivotal role in protecting motor neurons from damage.
A study estimates that brain and nervous system disorders in the US may cost as much as $1.2 trillion annually, with complex genetics contributing significantly to the disease burden. Genetic factors are believed to play a major role in these disorders, particularly those with high heritability rates.
A protein called huntingtin is critical for normal neuronal transportation, but a defective version causes physical blockage and binding interference, leading to neuronal damage. The study supports the hypothesis that blockage of neuronal transportation contributes to neurodegenerative diseases.
Neurons in the striatum, responsible for emotions and movement, are selectively killed in Huntington's disease due to abnormally high calcium levels caused by mutant huntingtin protein. This discovery opens new areas for treatment of the disease.
Scientists have successfully silenced mutant genes without affecting normal gene copies using RNA interference, a promising approach for treating diseases like Machado-Joseph disease, Huntington's, and Alzheimer's. This breakthrough technique has the potential to selectively turn off disease-causing genes, preserving essential normal g...
A team of scientists discovered that small heat-shock proteins play a key role in delaying both aging and age-related diseases such as Alzheimer's, Huntington's, and Parkinson's. The proteins inhibit protein aggregation, suggesting a molecular link between the two conditions.
Researchers found that small toxic molecules triggering cell damage in degenerative diseases have a similar structure. This discovery implies that these molecules are suitable targets for new drugs or vaccines that could halt progression of many degenerative diseases.
Researchers found that intermittent fasting reduced degeneration of nerve cells and improved glucose regulation in mice with mutant huntingtin. This suggests that fasting may forestall the development of Huntington's disease in humans.