Keyword: huntingtons
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SAN DIEGO – Sometimes, scientific breakthroughs occur when researchers aren’t exactly looking for them. While attempting to better understand the function of a protein in connective tissue cells, UC San Diego School of Medicine scientists found a way to transform multiple types of cells into neurons. This discovery has led to the development of a treatment that eliminates symptoms of Parkinson’s disease in mice. The protein researchers were studying, called PTB, is known for its general role in activating or deactivating genes within a cell. In an attempt to better understand how PTB contributes to cell function, researchers silenced the...
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An international team of scientists have confirmed the discovery of a major cause of dementia, with important implications for possible treatment and diagnosis. Professor Garth Cooper from The University of Manchester, who leads the Manchester team, says the build-up of urea in the brain to toxic levels can cause brain damage - and eventually dementia. The work follows on from Professor Cooper's earlier studies, which identified metabolic linkages between Huntington's, other neurodegenerative diseases and type-2 diabetes. The team consists of scientists from The University of Manchester, the University of Auckland, AgResearch New Zealand, the South Australian Research and Development Institute,...
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Watertown, MA—Scientists at Boston Biomedical Research Institute (BBRI) and the University of Pennsylvania have found that combining two chemicals, one of which is the green tea component EGCG, can prevent and destroy a variety of protein structures known as amyloids. Amyloids are the primary culprits in fatal brain disorders such as Alzheimer's, Huntington's, and Parkinson's diseases. Their study, published in the current issue of Nature Chemical Biology (December 2009), may ultimately contribute to future therapies for these diseases. "These findings are significant because it is the first time a combination of specific chemicals has successfully destroyed diverse forms of amyloids...
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HUNTINGTON'S DISEASE (HD) is caused by the aggregation of a different type of proteins. Some proteins have a repeat of a single amino acid (glutamine, often abbreviated as "Q"). These poly-Q repeats, if long enough, form aggregates which cause HD. We are studying the structure of poly-Q aggregates as well as predicting the pathway by which they form. Similar to Alzheimer's Disease, these HD studies, if successful, would be useful for rational drug design approaches as well as further insight into how HD aggregates form kinetically (hopefully paving the way for a method to stop the HD aggregate formation).
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Mayo Clinic researchers have discovered a protein interaction that may explain how the deadly Huntington's disease affects the brain. The findings, published in and featured on the cover of the current issue of Human Molecular Genetics, show how the mutated Huntington's protein interacts with another protein to cause dramatic accumulation of cholesterol in the brain. "Cholesterol is essential for promoting the connection network among brain cells and in maintaining their membrane integrity. Both the level of cholesterol and its delivery to the proper locations in the cell are essential for the survival of neurons," explains Mayo Clinic molecular biologist Cynthia...
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At the Institut Curie, CNRS and Inserm researchers have shown that cysteamine, which is already used to treat a rare disease called cystinosis, prevents the death of neurons in Huntington’s disease. Like Alzheimer’s and Parkinson’s, Huntington’s disease, is characterized by the abnormal death of neurons. Cysteamine raises neuronal levels of BDNF protein, a trophic factor which is depleted in Huntington’s disease, and by assaying BDNF in the blood it is possible to evaluate the effect of treatment. If other studies confirm these results, cysteamine could soon be used to treat Huntington’s disease, and BDNF could serve as a biomarker of...
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Scientists makes good use of its surprising similarity to humans BAR HARBOR, MAINE -- Nov. 9, 2006 Who would have guessed that the lowly sea urchin, that brain-less, limb-less porcupine of the sea, would be the star of a multi-million dollar, worldwide effort to map out every letter of its genetic code? Or that the information gathered in that effort may eventually lead to new treatments for cancer, infertility, blindness, and diseases like muscular dystrophy and Huntington's Disease?James Coffman, Ph.D., of the Mount Desert Island Biological Laboratory in Bar Harbor was one of the scientists who helped decode the 814...
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An enzyme known to be critical for the repair of damaged cells and the maintenance of cellular energy may be a useful target for new strategies to treat Huntington's disease (HD) and other disorders characterized by low cellular energy levels. In the August issue of Chemistry & Biology, a research team from the MassGeneral Institute for Neurodegenerative Disease (MIND) describes their discovery of a novel inhibitor of Poly (ADP-ribose) polymerase (PARP1) and their findings that PARP1 inhibitors can protect HD-affected cells from damage in laboratory assays. "While PARP1 is essential for the repair of damaged DNA, we also know that,...
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GAINESVILLE, Fla., June 14 (SPX) -- Regenerative medicine scientists at the University of Florida's McKnight Brain Institute have created a system in rodent models that for the first time duplicates neurogenesis - the process of generating new brain cells - in a dish. Writing in today's (June 13) Proceedings of the National Academy of Sciences, researchers describe a cell culture method that holds the promise of producing a limitless supply of a person's own brain cells to potentially heal disorders such as Parkinson's disease or epilepsy. "It's like an assembly line to manufacture and increase the number of brain cells,"...
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Researchers at Rush University Medical Center, Chicago, and Ceregene Inc., San Diego, have successfully used gene therapy to preserve motor function and stop the anatomic, cellular changes that occur in the brains of mice with Huntington’s disease (HD). This is the first study to demonstrate that, using this delivery method, symptom onset might be prevented in HD mice with this treatment. Results of the study were published in the Proceedings of the National Academy of Sciencesof the United States, June 13, 2006. “This could be an important step toward a disease modifying therapy,” says co-author Jeffrey H. Kordower, Ph.D., director...
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