Articles tagged with Huntingtons Disease
Researchers have successfully lowered levels of mutant huntingtin protein in patients with Huntington disease, making the treatment safe and well-tolerated. The trial enrolled 46 patients and found significant decreases in disease-causing protein levels without serious adverse reactions.
A commonly used blood pressure medication has shown potential in slowing the progression of neurodegenerative diseases such as Parkinson's and dementia. The study, published in Nature Communications, found that felodipine induced autophagy in mice with Huntington's disease and zebrafish models of dementia.
A UK researcher has been awarded a fellowship to collaborate with Chinese scientists on a novel treatment strategy for Huntington's disease. The project aims to establish sustainable research links between the UK and China, with the goal of accelerating the development of an effective therapy for this devastating neurological disorder.
Researchers found that inducing jet lag in fruit fly models of Huntington disease protected the flies' neurons. The team identified a circadian clock-controlled gene that also protected the brain when knocked down. This study suggests that targeting this gene could potentially slow the progression of neurodegenerative diseases.
Researchers estimated how common carriers of intermediate and pathological range polyglutamine disease-associated gene variants were among the general population using data from five European studies. The study found that approximately 2% of participants carried intermediate range variants and 1% carried pathological range variants.
University of California, Irvine researchers will receive $6 million in funding to support the development of a new treatment for Huntington's disease. The two-year study aims to achieve FDA approval and begin first-in-human clinical trials.
Researchers from the University of Konstanz develop an RNA-based inducible system for switching on genes in C. elegans, closing a significant gap in the research on genetic switches. The new approach establishes a novel inducible disease model for Huntington's disease, opening up new opportunities for research and application.
New guidelines developed by an international expert panel emphasize the importance of treating neuropsychiatric disorders in HD patients. The guidelines provide general, behavioral, and pharmacologic recommendations for managing symptoms such as agitation, anxiety, apathy, psychosis, and sleep disorders, and highlight the need for regu...
Researchers have developed a reliable method to measure the length of the Huntingtin gene, which causes Huntington's disease. The new technique reduces analysis time from over five hours to just five minutes, significantly speeding up diagnosis. This breakthrough has potential applications for other trinucleotide diseases.
A new study by researchers from the University of Copenhagen reveals that glial cells play a crucial role in the development of Huntington's disease. The study found that glial cell maturation is severely impaired in patients with the disease, leading to behavioral and motor changes.
Scientists have gained a clearer understanding of what happens in the brains of people with Huntington's disease, highlighting the crucial role of glial cells. Glial cell dysfunction is believed to drive neuronal loss and disease progression, leading to motor control problems and cognitive decline.
Researchers found that greater cognitive activity throughout life delays the appearance of symptoms and reduces grey matter loss in the brain. Participants with higher educational levels, language proficiency, and cognitive stimulation showed better neuropsychological test scores and slower disease progression.
Researchers developed an antisense oligonucleotide therapy to halt or reverse SCA7-induced vision loss. Studies in mice and humans show promise for treating the rare condition with fewer side effects than existing medications.
Researchers at the University of British Columbia have discovered that reducing mutated Huntington disease protein can restore cognitive and psychiatric impairments in mice. This breakthrough offers new hope for preventative treatment, as approximately one in seven thousand people in Canada suffer from the debilitating disease.
A new study finds that a simple blood test can detect early changes caused by Huntington's disease, even before brain scans can pick up signs. The test measures two biomarkers, NfL protein in blood and mutant huntingtin protein in brain fluid, to track the disease's progression.
A research team at Max Delbrück Center identified tiny huntingtin protein fibers that precede larger deposits in Huntington's disease, enabling prediction of disease onset months in advance. These findings hold promise for diagnosis and potential new treatments by testing pharmaceutical substances against the fibers' harmful activity.
Researchers at the University of Tennessee suggest that a genetic mutation may cause Huntington's disease to appear earlier in life. A new drug, Panobinostat, may regulate gene expression and prevent disease progression.
A direct link has been discovered between protein aggregation in nerve cells and the regulation of gene expression in Huntington's disease. The study found that impaired autophagy leads to accumulation of misfolded proteins, including AGO2, which disrupts cell function and signal pathways.
A new system has been identified that could reduce neurodegeneration in Huntington's disease by blocking the accumulation of toxic protein aggregates. The system involves a protein called UBR5, which promotes the degradation of mutant huntingtin, leading to a decrease in neurotoxic effects.
Scientists have developed a new drug discovery system that can specifically target phosphatase enzymes, which were previously considered undruggable. The system identified a molecule that successfully targeted a phosphatase to reduce accumulation of disease-associated proteins in mouse brains.
Researchers at McMaster University have discovered a unique type of signaling from damaged DNA that triggers huntingtin activity in DNA repair. This signaling is defective in Huntington's disease and can be restored with a molecule called N6-furfuryladenine, reversing symptoms and restoring mutant huntingtin protein levels to normal.
A team of scientists developed a high-throughput approach to integrate laboratory experiments, literature data, and network analysis to study Huntington's disease. The approach revealed that changes in inflammation, cell architecture, and calcium signaling drove the disease forward, while counteracting these changes improved health.
A new study found a hitherto unknown error in the transport of glutamine between astrocytes and neurons in mice with Huntington's disease. The researchers believe this area holds potential for developing a future treatment.
A new study reveals that the brain relies on an exquisite balance between two populations of neurons in the striatum to control movement. The findings could help researchers develop new treatments for Parkinson's and Huntington's diseases by understanding how movement gets translated into desired action.
A study published in Nature Communications has found an unexpected overlap between the causes of rheumatoid arthritis and Huntington's disease. The researchers developed a novel algorithm to analyze epigenetic data, identifying new cell signaling pathways and potential treatment options for both conditions.
Researchers found that a defective protein in Huntington's disease disrupts the mTORC1 pathway, leading to cardiac problems. Adjusting this pathway improved heart function in experimental animals, offering new insights into the biology of the disease.
Researchers found mice engineered with Huntington's disease have excessive mitochondrial iron accumulation, leading to dysfunction and neuronal death. This study identifies a pathway for the neurodegenerative disease and has implications for related disorders like Parkinson's and Alzheimer's.
Researchers at UCLA have developed a new method that enables them to observe astrocytes' influence on nerve-cell communication in real time, shedding light on their role in neurological disorders such as Alzheimer's and Huntington's disease.
A new study identifies alterations in functional connectivity across the whole brain in carriers of the Huntington's disease gene mutation, associated with motor and cognitive function. The study also shows significant associations between the extent of the degree of gene mutation and measures of brain connectivity.
Researchers at the University of British Columbia found that restricting food access to a six-hour window per day stimulated autophagy and lowered mutant huntingtin protein levels in mice with Huntington disease. This suggests a potential new treatment approach for the disorder, which could be complementary to existing therapies.
Researchers have developed a safer and more specific CRISPR/Cas9 system to treat Huntington's disease, a neurodegenerative disorder caused by a defective gene. The new technique successfully inactivates the mutant gene and reduces toxic protein synthesis, offering hope for a potential cure.
Scientists have decoded the three-dimensional molecular structure of the healthy human huntingtin protein, enabling its functional analysis. This breakthrough could contribute to the development of new treatments for Huntington's disease.
Scientists have discovered that the Huntington's gene is toxic to cancer cells due to its repetitive RNA sequences, which can be harnessed for a novel cancer treatment approach. Researchers hope to develop a short-term treatment to kill cancer cells without causing neurological issues associated with Huntington's disease.
Scientists at WashU Medicine have transformed skin cells from patients with Huntington's disease into brain cells, allowing them to study the degenerative disorder. The resulting neurons exhibited 'symptoms' of the disease, including DNA damage and cell death, which were prevented by correcting malfunctioning genes.
Scientists at Rockefeller University discovered early abnormalities in human embryonic stem cells with Huntington's disease, suggesting the disorder originates much earlier than previously thought. The study implies that existing treatments may do more harm than good and necessitates a new approach to treating the disease.
A new study found that brains of people with Huntington's disease (HD) and Parkinson's disease (PD) show similar responses to a lifetime of neurodegeneration, despite being distinct diseases. Most genes related to immune response and inflammatory pathways are common in both diseases.
Researchers at Boston University School of Medicine discovered a possible biomarker for Huntington's disease, which may help evaluate treatment effectiveness and potentially delay disease onset. The study found altered levels of microRNAs in individuals carrying the HD gene mutation twenty years before symptoms appear.
Researchers found that restricting food availability to a set schedule improved motor activity and sleep quality in mice with Huntington's disease. These findings suggest that eating on a strict schedule could improve quality of life for patients with neurodegenerative diseases.
Researchers identify build-up of urea in brain as major cause of dementia, with potential for early diagnosis and treatment. The study suggests that high brain urea levels may be a pivotal role in all types of age-related dementias.
A study by Lancaster University found that people with Huntington's Disease welcome discussing assisted death but feel restricted from doing so. The participants feared prolonged suffering and believed they should have the right to make decisions about their own deaths.
A study found that a cancer medication can improve symptoms of Huntington's disease in mice, including increased mobility and longer lifespan. The drug targets mitochondrial health and protein removal, also relevant to other neurodegenerative disorders.
Don Cleveland and Peter Walter are recognized for their pioneering work in cell biology, with focus on protein synthesis and chromosome movement. The Breakthrough Prize acknowledges their contributions to advancing our understanding of cellular mechanisms.
Researchers trained sheep to recognize celebrity faces and found they could identify their handler's face without prior training. The study suggests sheep have advanced face recognition abilities, comparable to those of humans and monkeys.
A team of researchers has created a model to analyze short tandem repeats in the human genome, which are implicated in about 30 harmful conditions. The model predicts the frequency and mutation rate of repeats, providing insights into constraints that may narrow down disease signals.
A new version of the CRISPR-Cas9 gene editing technique has been developed to correct molecular mistakes that lead to microsatellite repeat expansion diseases. The approach eliminated 95% or more of RNA foci linked to myotonic dystrophy and other diseases in laboratory models and patient-derived cells.
The University of Plymouth has developed HDNetDB, the first freely available data network for scientists researching Huntington's disease. This resource links various cellular processes in a molecular network and provides a more holistic view of the disease.
A new study from the University of California, Berkeley found that dementia patients tend to die sooner when cared for by family members with depression or anxiety. The study tracked 176 patients and their caregiver mental health over several years, revealing a strong link between patient survival and caregiver well-being.
Researchers have identified a genetic modifier associated with the rate of progression in Huntington's disease. The study used high-quality data from two cohorts and found a significant result linked to the MSH3 gene.
Scientists used CRISPR/Cas9 gene editing to reverse Huntington's disease pathology and motor symptoms in a mouse model. The treatment delivered enzymes to brain cells, reducing toxic protein aggregates and improving motor abilities.
A new blood test has been identified to predict the onset and track the progression of Huntington's disease. The test measures neurofilament levels in the blood, which increase throughout the course of the disease, even in carriers of the genetic mutation.
Researchers have discovered a molecular mechanism behind prion protein's role in neurodegenerative diseases, including Creutzfeldt-Jakob and Alzheimer's. The study found that copper biases the prion protein towards its 'off' state, which reduces toxic signals to nerve cells.
Researchers found that abnormal proteins in Alzheimer's, Parkinson's, and Huntington's diseases cause the same type of vesicle damage, potentially leading to effective treatments for multiple neurodegenerative diseases.
Researchers found that traffic jams in the nucleus kill brain cells in Huntington's disease. Drugs clearing up these disruptions restored normal transport and saved cells.
A new special collection in Disease Models & Mechanisms (DMM) explores the intersection of models and mechanisms to therapies for neurodegenerative disorders. The collection includes articles on induced pluripotent stem cell models, antisense oligonucleotide therapy for spinal muscular atrophy, and more.
Researchers use computer simulations to show how N-terminal sequence encourages aggregation of huntingtin protein fragments while polyproline inhibits it. This discovery offers a new target for drug development to halt Huntington's disease progression. The study also highlights the involvement of the cytoskeleton in the disease mechanism.
A new study found a link between neurological birth defects in infants and several neurodegenerative diseases, including Alzheimer's, Parkinson's, and Huntington's. Researchers also identified a medicine that could treat these diseases by reducing misfolded proteins.
Researchers at EPFL develop synthetic methods to introduce chemical modifications on huntingtin, reducing its toxicity and aggregation. The study reveals key findings on the relationship between post-translational modifications and huntingtin structure, function, and toxicity.
A study published in JCI reveals that mutant HTT protein and DISC1 form a complex that compromises its functions, leading to disruptions in downstream pathways. Normalizing the activity of this protein complex improves cognitive symptoms in HD mice, providing insights into DISC1's role in mental illness.
Researchers have identified early biomarkers of Huntington's disease in a five-year-old HD sheep, revealing metabolic changes prior to physical symptoms. These findings suggest that the disease affects important bodily processes before they become apparent, and could lead to the development of new treatments.
A study by Duke Health researchers has identified a shared root cause of Huntington's disease with Alzheimer's and other neurodegenerative diseases. The team found that a biochemical explanation for the breakdown of quality control processes in Huntington's disease, which can be restored by chemically inhibiting CK2, holds promise for ...