Keyword: prion
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The seeds of Alzheimer’s disease can be transmitted through medical procedures, scientists have found, leading experts to call for the monitoring of blood transfusions from the elderly and those with a family history of dementia. In 2015, researchers at University College London discovered that people who developed Creutzfeldt-Jakob disease (CJD) following treatments with human growth hormone also showed signs of Alzheimer’s in their brains after death. (Please see full article at link)
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The sickness spread at funerals. The Fore people, a once-isolated tribe in eastern Papua New Guinea, had a long-standing tradition of mortuary feasts — eating the dead from their own community at funerals. Men consumed the flesh of their deceased relatives, while women and children ate the brain. It was an expression of respect for the lost loved ones, but the practice wreaked havoc on the communities they left behind. That’s because a deadly molecule that lives in brains was spreading to the women who ate them, causing a horrible degenerative illness called “kuru†that at one point killed 2...
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In the late 1950s, some 2 percent of the Fore tribe from Papa New Guinea died each year from a rare neural disease known as kuru. The mad cow-like disease was spread by the tribe's now-retired practice of eating the brains of their deceased relatives. A new study, however, has identified a gene adaptation that protected a small subset of the Fore population from kuru and other similar diseases. Kuru and other similar diseases are characterized by the proliferation of misshapen neural proteins called prions. These invaders stick together in infected brains, forming plaque-like polymers that slowly suffocate neural pathways...
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As if it weren't bad enough that deadly prions can survive boiling and radiation, now comes word that aerosolized forms of the pathogen can enter the nose and find their way to the brain, with fatal consequences. Prions, you may recall, were the reason you avoided beef in Europe in the 1990s. They triggered the infamous mad cow disease epidemic in the U.K., which spread to the rest of Europe and other parts of the world. Prions are proteins that all animals produce, but sometimes, toxic mutant versions are made. These malformed versions can cause ..."
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Non-infectious form of prion protein could cause brain degeneration. Prion proteins may react with amyloid-(beta) peptides inside the brain cells of Alzheimer's patients.Thomas Deerinck NCMIR/Science Photo Library Non-infectious prion proteins found in the brain may contribute to Alzheimer's disease, researchers have found. The surprising new results, reported this week in Nature1, show that normal prion proteins produced naturally in the brain interact with the amyloid-(beta) peptides that are hallmarks of Alzheimer's disease. Blocking this interaction in preparations made from mouse brains halted some neurological defects caused by the accumulation of amyloid-(beta) peptide. It was previously thought that only infectious prion...
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Even vegetarians may not be safe from 'mad cow' prions 10:34 26 June 2008 NewScientist.com news service Ewen Callaway Fancy a dose of prions with your vegetables? A new study suggests that infectious prions - thought to be the causative agents in mad cow disease and human vCJD – can survive wastewater decontamination and wind up in fertiliser, potentially contaminating fruit and vegetables. The prions would be present in such low quantities that they are unlikely to pose a health threat, but as a precaution, "we should prevent the entry of prions into wastewater treatment plants," says microbiologist Joel Pedersen,...
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Transmitting Prion Diseases In Milk ScienceDaily (Apr. 8, 2008) — Scrapie can be transmitted to lambs through milk, according to new research. The study provides important information on the transmission of this prion-associated disease and the control of scrapie in affected flocks. Scrapie is a fatal neurodegenerative disease of sheep and goats. Clinical signs include itchiness, head tremor, wool loss and skin lesions as well as changes in behaviour and gait. Timm Konold and colleagues from the Veterinary Laboratories Agency in Weybridge, UK, investigated the transmission of scrapie by feeding milk from scrapie-affected ewes to lambs that are genetically susceptible...
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Scientists at Harvard University have developed a computer model that, for the first time, can fully map and predict how small proteins fold into three-dimensional, biologically active shapes. The work could help researchers better understand the abnormal protein aggregation underlying some devastating diseases, as well as how natural proteins evolved and how proteins recognize correct biochemical partners within living cells. The technique, which can track protein folding for some 10 microseconds -- about as long as some proteins take to assume their biologically stable configuration, and at least a thousand times longer than previous methods -- is described this week...
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Scientists have long eyed an enigmatic protein called PrP as the culprit behind deadly prion diseases, including "mad cow" disease. Now they've identified one of its functions in healthy animals: helping certain stem cells in bone marrow divide and survive. It's not clear what the find means for the protein's behavior in the brain, but prion experts say that uncovering any function for PrP is a big step forward. PrP is found throughout the body, and it's especially abundant in the brain. In rare cases, PrP can misfold and clump together, potentially leading to fatal prion diseases. But scientists have...
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AP FOOD AND FARM WRITER WASHINGTON -- Two cases of mad cow disease in Texas and Alabama seem to have resulted from a mysterious strain that could appear spontaneously in cattle, researchers say. Government officials are trying to play down differences between the two U.S. cases and the mad cow epidemic that has led to the slaughter of thousands of cattle in Britain since the 1980s. It is precisely these differences that are complicating efforts to understand the brain-wasting disorder, known medically as bovine spongiform encephalopathy, or BSE for short. "It's most important right now, till the science tells us...
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While newly published research confirms that under laboratory circumstances prion-protein can be absorbed across the gut, it also shows that this is unlikely to occur in real life. In addition, the results show that the places in the gut that do take up these disease-associated proteins are different from the locations where infectivity is known to be amplified. The findings will be published in the Journal of Pathology. Since the outbreak of BSE in cattle and vCJD in humans, scientists have struggled to make sense of how an abnormal variation of a normal protein can trigger an infectious disease. Some...
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BOISE, Idaho (AP) -- State and federal health officials are trying to get to the bottom of nine reported cases of suspected sporadic Creutzfeldt-Jakob disease, a naturally occurring form of the fatal brain-wasting illness. "One thing is very clear in Idaho -- the number seems to be higher than the number reported in previous years," said Dr. Ermias Belay, a CJD specialist with the federal Centers for Disease Control and Prevention (CDC). "So far, the investigations have not found any evidence of any exposure that might be common among the cases." Normally, sporadic CJD strikes about one person in a...
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A new case of Mad Cow disease has been reported by the Dept.of Agriculture in the U.S....BREAKING.......
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Scientists are reporting that, for the first time, they have made an artificial prion, or misfolded protein, that can, by itself, produce a deadly infectious disease in mice and may help explain the roots of mad cow disease. The findings, being reported today in the journal Science, are strong evidence for the "protein-only hypothesis," the controversial idea that a protein, acting alone without the help of DNA or RNA, a cousin of DNA, can cause certain kinds of infectious diseases. The concept was introduced in 1982 by Dr. Stanley Prusiner, a neurology professor at the University of California, San Francisco,...
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Research suggests sick animals may not show symptoms Below the drumbeat of reassurances from government and the cattle industry that the meat supply remains safe despite this one case of mad cow disease, a small universe of scientists working on a family of related illnesses is finding disturbing evidence to the contrary. Several studies, including research at a government laboratory in Montana, continue to spark questions about human susceptibility not only to mad cow, but also to sister diseases such as chronic wasting disease, which mainly affects deer and elk, and scrapie, which infects sheep. Mice research and clusters of...
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Mad Cow Disease What the Government Isn't Telling You! What is Mad Cow Disease? Mad Cow Disease is the common term for Bovine Spongiform Encepholopathy (BSE), a progressive neurological disorder of cattle which can be transmitted to other species, including humans. In humans, it is called Creutzfeldt-Jakob Disease, after the two doctors who first described the symptoms of the disease. The disease in cattle is called Bovine Spongiform Encepholopathy because this form of the disease occurs in cows (therefore, the term bovine), it causes a sponge-like destruction of the brain (therefore, the term spongiform encepholopathy - enceph means brain and...
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Following is a synopsis of the current knowledge about scrapie and resource information. Causative agent, transmission:Scrapie is a tranmissable spongiform encephalopathy widely thought to be caused by a misfolded prion protein that accumulates in the brain. However, there are also two other theories--that the agent is a virus with unusual characteristics, or a virino, a small, noncoding regulatory nucleic acid coated with a host-derived protective protein. According to Dr. Katherine O'Rourke, a USDA research microbiologist in Pullman, Washington, "Infection is believed to be caused by ingestion of that misfolded protein (through the placenta and placental fluids) by the lamb or...
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