Articles tagged with Huntingtons Disease
Scientists from Scripps Research Institute have established conclusively that an activating protein called Rhes plays a pivotal role in focusing toxicity of Huntington's in the striatum. Deleting Rhes significantly reduces behavioral problems in animal models of the disease.
Researchers will investigate mTOR's role in brain function and regulation using various techniques. Understanding its function in the striatum could lead to new therapies for neurodegenerative diseases.
A new transgenic nonhuman primate model of Huntington's disease has been developed, showing similarity to humans in progressive neurodegeneration and motor control decline. The study reveals cognitive and motor impairments emerging at 16 months of age, and dystonia and signs of neurodegeneration on brain imaging at 24 months.
Researchers identified a gene variant outside the huntingtin gene that affects disease onset timing. The variant delays symptoms when on the normal protein copy, while accelerating them on the mutated protein copy.
A closer look at the DNA surrounding the Huntington's disease (HD) gene reveals critical regions controlling its expression. Changes in these regions can delay or accelerate the disease, with some individuals receiving protection from the mutant gene.
A team of scientists discovered CRMP1, a protein that acts as a 'chaperone' to prevent misfolding of the toxic huntingtin protein. In healthy brains and tissues, CRMP1 is present in higher amounts than in those affected by Huntington's disease.
A novel computational strategy identified CRMP1 as a factor that suppresses misfolding and aggregation of the huntingtin protein in Huntington's disease. CRMP1 overexpression reduces huntingtin aggregation and toxicity, while reduced levels increase toxicity.
Researchers have identified significant vascular changes in Huntington's disease brains, finding mutant huntingtin in blood vessels and increased permeability of the blood-brain-barrier. These findings suggest that healthy cells can be infected by the mutant protein, offering new potential therapeutic targets.
A team of researchers from Plymouth University is studying a protein called Bim, which causes cell death in the brain and regulates autophagy and apoptosis. They aim to understand how Bim levels increase in Huntington's disease and test the effectiveness of a Bim-derived peptide in treating the disease.
A new ultra-sensitive test can detect mutant huntingtin protein in the cerebrospinal fluid of HD patients, predicting disease severity. The test has the potential to guide clinical decisions and monitor treatment efficacy.
Researchers are developing a biologically plausible model of the whole system to predict outcomes of future experiments and provide more information about the brain's ability to learn new motor movements. The goal is to understand how people execute, control and learn movements to lead to new treatments for Huntington's disease.
Researchers at Boston University School of Medicine identified a specific genetic signal, miR-10b-5p, that strongly correlates with disease severity and extent of neuronal death in Huntington's disease. This discovery may provide a faster and more effective way to determine the effectiveness of treatments.
Researchers at Scripps Research Institute have identified a crucial pathway that shields cells against death caused by environmental stress. The study found that Rheb inhibits protein synthesis by amplifying the phosphorylation of eIF2α, conserving cell resources during challenging conditions.
Researchers from Inserm confirm triheptanoin's efficacy in treating Huntington's disease by restoring normal brain energy metabolism. A pilot clinical trial with ten patients showed significant improvements, paving the way for a larger-scale trial.
Researchers found improvement in movement and motor skills in people with Huntington's after one month of triheptanoin therapy. The study showed that the synthetic oil could improve brain metabolism in early stages of the disease.
A novel treatment approach for Huntington's disease shows promise by adjusting key signaling protein levels in experimental animals. The study improves motor function, reduces brain abnormalities, and promotes metabolic health in mice bred to model the disorder.
A new study from The Scripps Research Institute suggests a drug compound can benefit not only parents but also their children by changing genetic expression, leading to improved memory and motor skills in offspring with Huntington's disease. This breakthrough discovery offers promising potential for treating the inherited disorder.
Researchers discovered a link between blood pressure hormone angiotensin and psychosis, while finding that increased cysteine levels may alleviate symptoms in people with Huntington's disease. Additionally, drugs targeting AMPA receptors improved social behaviors in autistic mice.
Researchers found that huntingtin protein activates signaling by mTORC1, leading to premature disease onset and worsening symptoms. The study offers new target for drug development, potentially preventing neurodegeneration through reduced mTORC1 activation.
Scientists applied iPS cell technology to a transgenic nonhuman primate model of Huntington's disease, developing cellular features of the condition and discovering potential therapies for oxidative stress. This approach could aid in the discovery and evaluation of other treatments for the disorder.
A study suggests that the hippocampus plays a crucial role in the development of Huntington's disease cognitive decline. Researchers found increased expression of p75NTR in the hippocampus, leading to reduced dendritic spine density and memory defects.
A study at Oak Ridge National Laboratory reveals structural differences between normal and diseased forms of the huntingtin protein, which is involved in Huntington's disease. The researchers used neutron scattering to compare the structures over time, finding key discrepancies that support a growing focus on amyloid disorders.
A recent Duke University study has found that the mutated Huntington's disease protein is crucial for normal brain development and synaptic circuitry in early life. The research suggests that faulty connections may be the root cause of neurodegenerative disorders like Alzheimer's, with potential implications for treatment strategies.
Scientists have discovered that a defect in the huntingtin gene impairs mitochondria, leading to brain cell death in Huntington's disease. The study found that brain cells rely heavily on their mitochondria, making them vulnerable to disruption.
The Structural Genomics Consortium and CHDI Foundation have entered into an open-access research collaboration to discover new drug targets for Huntington's disease. The partnership, which will make research tools freely available without restriction, aims to accelerate discovery of new medicines for the neurodegenerative disorder.
A recent study published in Annals of Neurology reports that healthy human tissue grafted to the brains of patients with Huntington's disease developed signs of the illness, several years after grafting. The study has profound implications on the understanding of the disease and how to treat it.
Blocking extrasynaptic NMDA receptors may improve motor learning, coordination and prevent cell death in animal models of Huntington disease. This finding could lead to new treatment avenues for neurodegenerative conditions such as Alzheimer's disease and traumatic brain injury.
A novel RNAi therapy successfully blocks production of the dysfunctional huntingtin protein, causing Huntington's disease. The treatment reduces mutant Htt levels and disease symptoms in a mouse model without causing neurotoxicity.
A study published in Neural Regeneration Research found that genetic diagnosis is crucial in ruling out Huntington's chorea. The analysis incorporated clinical symptoms, imaging examinations, and gene diagnosis, suggesting that a combination of these factors is necessary for an accurate diagnosis.
Researchers used a genetic approach to find that cortical neurons play a key role in initiating the disease, while shutting down mutant huntingtin in both sets of cells corrected symptoms. The study suggests new targets for therapeutic drugs to slow the devastating disease.
Researchers found that protein aggregates in Huntington's disease are not toxic, but rather a defense mechanism for stressed brain cells. This discovery may lead to new therapeutic approaches by targeting the stress response instead of protein clusters.
Researchers at Michigan State University have developed a promising new treatment for Parkinson's disease using a molecular tweezer that prevents protein aggregation. The molecule, CLR01, speeds up protein reconfiguration and has shown success in slowing the first step of aggregation, paving the way for clinical trials.
A UCLA study found that increasing Kir4.1 levels in astrocytes improves walking and prolongs survival in a mouse model of Huntington's disease. The discovery could lead to new drug targets for treating the devastating disorder, which affects one in every 20,000 Americans.
Researchers identified cysteine deficiency as a cause of brain degeneration in Huntington's disease. A cysteine-rich diet slowed disease progression in mice, but its effectiveness in humans is unclear.
A study published in PLOS Genetics identified specific small segments of RNA that are highly expressed in Huntington's disease and may act as a mitigating factor, making them potential therapeutic targets. The researchers found that these microRNAs are present in higher quantities in patients with HD and may promote cell survival.
A large-scale protein interaction network for Huntington's disease has been identified, providing valuable insights into the disease's pathology. The network implicates the RhoGTPase signaling pathway, which affects cell motility, membrane dynamics, and cell attachment, offering potential therapeutic targets.
Huntington's disease is caused by an expansion in the polyglutamine tract of a protein called Huntingtin, leading to its misfolding and aggregation. Researchers have discovered that transient intermediate species called oligomers play a key role in neurotoxicity, rather than fibrillar aggregates. Modulating these oligomers through mole...
Researchers have discovered that naturally occurring gatekeeper sequences on either side of a key protein mutation in Huntington's disease can prevent the formation of toxic structures. This breakthrough offers new hope for understanding and treating the devastating neurodegenerative disorder.
Researchers at OHSU discovered a way to use small molecules to fix misfolded proteins, allowing them to function normally again. This technique has the potential to treat diseases such as cystic fibrosis, cataracts, and Alzheimer's disease.
Researchers have identified a new therapeutic target for Huntington's disease, the HDAC4 enzyme. Halving levels of HDAC4 in cells can delay aggregation of mutant huntingtin protein and rescue nerve cell function.
Researchers tested a drug that acts like growth-promoting protein BDNF and found it reduces degeneration and motor deficits in two mouse models of Huntington's disease. The findings suggest drugs that enhance BDNF action could be effective therapeutics for treating the disorder.
University of Adelaide researchers have identified a likely molecular pathway that causes neurodegenerative diseases such as Huntington's and Lou Gehrig's. The team found that RNA plays a key role in the development of these diseases, which share similar genetic mutation mechanisms.
A Mediterranean-type diet was not associated with a delay in the clinical onset of Huntington's disease, according to a study. High dairy consumption and caloric intake were found to increase the risk of phenoconversion. The study suggests that modifying specific components of the diet may help delay the onset of the disease.
A study has identified a metabolic network associated with Huntington's disease progression, allowing for predictive assessment of time to symptom onset. This discovery provides biomarkers for evaluating disease progression in carriers and supports the incorporation of this assessment into clinical trials.
Researchers used PET scanning to map changes in brain metabolism in Huntington's disease gene carriers, identifying a characteristic network of abnormalities. This finding enables the tracking of disease progression and will aid in evaluating the effects of new drugs before symptoms appear.
Researchers at the University of Leicester have discovered a potential molecular defence against Huntington's disease, a fatal neurodegenerative disorder. Glutathione peroxidase activity was found to be robustly protective in models of the disease.
Researchers at McMaster University have developed a method to measure the shape of the huntingtin protein in living cells, revealing a key clue to solving Huntington's disease. The team discovered that the mutant huntingtin protein causing disease changes shape and can be corrected with chemicals.
Researchers at Gladstone Institutes discover that individual neurons' ability to flush out toxic proteins, not the buildup itself, contributes to Huntington's disease progression. A newly developed technology allowed them to see how different types of neurons respond to mutant huntingtin protein over time.
A NIH-funded study found that rapidly removing defective proteins can help protect brain cells from death. Researchers developed a new technique to track protein turnover in neurons, revealing differences in how individual cells handle proteins. This discovery may lead to improved treatments for neurodegenerative diseases.
Researchers have discovered a biomarker that can track Huntington's disease progression using electrical activity in brain waves. The study found abnormalities in specific frequency bands prior to the onset of symptoms, suggesting a potential early indicator of the disease.
The First-HD study examines the efficacy and safety of SD-809, a novel drug for treating chorea associated with Huntington Disease. The trial aims to test the tolerability of the drug in patients who have not previously taken tetrabenazine.
Researchers successfully mobilize brain's native stem cells to replenish neurons lost in Huntington's disease. The study demonstrates the feasibility of a completely new concept to treat the disease by recruiting endogenous neural stem cells to regenerate cells, significantly extending survival of treated mice.
Researchers found that Cue1 factor contributes to marking defective proteins with molecular tag for degradation, enabling efficient removal. The CUE domain of Cue1 stabilizes ubiquitin chains, regulating formation of degradation signal.
Researchers at Lund University have prevented early symptoms of Huntington's disease, depression, and anxiety in mice by deactivating the mutated huntingtin protein. This discovery is a major breakthrough and may lead to more accurate treatments for this debilitating disease.
Researchers discover that expanded DNA regions in Fragile X-associated Tremor syndrome cause the production of an abnormal FMR1polyG protein, leading to neurodegeneration. The protein's translation is critical to elicit toxicity, and blocking its production can suppress neuron damage.
Researchers at the University of Manchester have made a breakthrough in developing an effective treatment for neurodegenerative diseases. They identified the molecular structure of the enzyme kynurenine 3-monooxygense (KMO) and discovered a compound that can inhibit its activity.
The NIH has awarded a $1.67 million grant to UT Dallas researchers, led by Dr. Santosh D'Mello, to investigate the connection between histone deacetylase-3 (HDAC3) and Huntington's disease. The study seeks to understand why specific brain cells degenerate in this devastating disorder.
A research team from KAIST solved the structure of Ataxin-1 and its binding partner Capicua, providing molecular details of their interaction. This discovery may lead to new therapeutic targets for treating Spinocerebella Ataxia Type 1 (SCA1) and related neurodegenerative diseases.
Scientists have identified a complex of three molecules that regulates the production of defective Huntingtin protein, a key contributor to Huntington's disease. By targeting this complex with pharmaceuticals, it may be possible to directly affect the production of defective proteins and treat the underlying causes of the disease.
The EU has announced €38 million funding for research into rare diseases, aiming to develop new diagnostics and treatments through global data sharing. Advances in DNA sequencing have brought personalized treatments closer, but scientists now need to collate data to identify genetic causes of diseases.