Keyword: huntingtonsdisease
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A preclinical study from Karolinska Institutet offers hope for treating severe neurodegenerative diseases with an existing drug. The study suggests that the leprosy drug clofazimine may be effective in the treatment of Huntington's disease. The research group examined whether existing drugs could reduce the toxicity of so-called polyQ proteins. These proteins are found in patients with certain hereditary neurodegenerative diseases, including Huntington's disease, for which there is no cure. Screening hundreds of drugs, they found that the leprosy drug clofazimine reduces the toxicity of polyQ proteins and restores mitochondrial function in zebrafish and worms. The finding supports the previous hypothesis...
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Could blood group anomaly explain Tudor king's reproductive problems and tyrannical behavior?DALLAS (SMU) – Blood group incompatibility between Henry VIII and his wives could have driven the Tudor king's reproductive woes, and a genetic condition related to his suspected blood group could also explain Henry's dramatic mid-life transformation into a physically and mentally-impaired tyrant who executed two of his wives. Research conducted by bioarchaeologist Catrina Banks Whitley while she was a graduate student at SMU (Southern Methodist University) and anthropologist Kyra Kramer shows that the numerous miscarriages suffered by Henry's wives could be explained if the king's blood carried the...
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FULL TITLE: Family's outrage at cops who violently arrested a father of two for 'appearing intoxicated while taking care of his kids'... but actually has a TERMINAL ILLNESS that makes him look that way Police officers in West Virginia brutally arrested a 39-year-old father of two who they say appeared intoxicated and was handling his kids roughly while walking them to the park. But his outraged family denies that 39-year-old Jeffrey Bane was under the influence on Sept. 6, when he was left choking in his own blood and pleading for help while he was pinned to the ground by...
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In a paper published in the July 11 online issue of Science Translational Medicine, researchers at the University of California, San Diego School of Medicine have identified two key regulatory proteins critical to clearing away misfolded proteins that accumulate and cause the progressive, deadly neurodegeneration of Huntington's disease (HD). The findings explain a fundamental aspect of how HD wreaks havoc within cells and provides "clear, therapeutic opportunities," said principal investigator Albert R. La Spada, MD, PhD, professor of cellular and molecular medicine, chief of the Division of Genetics in the Department of Pediatrics and associate director of the Institute for...
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Parents: Dying Girl, 7, Taunted by Neighbors in Trenton Updated: Friday, 08 Oct 2010, 7:24 AM EDT Published : Thursday, 07 Oct 2010, 11:56 PM EDT TRENTON, Mich. - Her family says 7-year-old Kathleen Edward is in the final stages of a degenerative brain disorder diagnosed as Huntington's Disease - the same disease which killed her mother, Laura, when she was only 24. A neighbor who is feuding with the family admitted to posting grim depictions of Laura and Kathleen on Facebook, and also has a coffin hitched to a pick-up truck in front of the house - which the...
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UCLA scientists have identified a molecular switch that prevents Huntington's disease from developing in mice. Published in the Dec. 24 edition of the journal Neuron, the discovery suggests a new approach to treating the genetic disorder, which ultimately leads to death in as little as 10 years. Affecting one out of every 10,000 Americans, Huntington's progressively deprives patients of their ability to walk, speak, think clearly and swallow. People who inherit the disorder don't show symptoms until mid-life, after many have had children and unknowingly passed on the disease. Currently, there is no effective treatment to prevent the onset or...
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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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Clioquinol inhibits action of the CLK1 aging gene, may alleviate Alzheimer'sRecent animal studies have shown that clioquinol – an 80-year old drug once used to treat diarrhea and other gastrointestinal disorders – can reverse the progression of Alzheimer's, Parkinson's and Huntington's diseases. Scientists, however, had a variety of theories to attempt to explain how a single compound could have such similar effects on three unrelated neurodegenerative disorders. Researchers at McGill University have discovered a dramatic possible new answer: According to Dr. Siegfried Hekimi and colleagues at McGill's Department of Biology, clioquinol acts directly on a protein called CLK-1, often informally...
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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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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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