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| | BMBL Section VII-D - Agent Summary Statements / Prions |
 | | Perhaps the most prudent approach is to study BSE prions in a Biosafety Level 2 or 3 facility depending on the samples to be studied, as noted above for human prions (i.e., brain, spinal cord). |  | | BSE prions are likewise manipulated at Biosafety Level 2 or 3, due to the possibility that BSE prions have been transmitted to humans in Great Britain and France. |  | | Mice and hamsters are the experimental animals of choice for all studies of prion disease. |
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http://www.cdc.gov/od/ohs/biosfty/bmbl4/bmbl4s7d.htm
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| | Molecular Evolution of Prions |
 | | Therein lies the interest of the similarities between human (and ape) prions and the prion of cattle -- similarities which occur nowhere else in the family tree, and significantly, not in sheep. |  | | The disturbing link between the prions that cause Bovine Spongiform Encephalopathy (BSE) in cattle and Creutzfeld-Jakob disease (CJD) in humans comes from a study of the evolutionary relationships of prions in a wide variety of mammals. |  | | Only after seventy weeks (which is late middle-age for a mouse) do things start to go wrong, according to Dr Suehiro Sakaguchi and his colleagues from the Nagasaki University School of Medicine in Japan, who report their findings in the April 11, 1996 Nature. |
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http://www.mad-cow.org/~tom/prion_evol.html
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| | Prion Page |
 | | This study is aimed at looking at the prion diseases from a molecular point of view, taking into account the genetic as well as the transmissible and sporadic aspect of the diseases. |  | | The study of kuru is immensely important for the understanding of these diseases, providing the most extensive clinical experience of acquired prion disease in humans, the epidemiology of kuru clearly indicates the infectious, but non-contagious, properties of prions and provides extensive evidence that vertical transmission from pregnancies does not occur. |  | | Their work involved the prion propagation using mice that expressed human and chimeric PrP transgenes. |
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http://www.fortunecity.co.uk/roswell/psychic/24/prionpage/Project.htm
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| | Prion Diseases |
 | | Using this technique it is very clear that the recent outbreaks of new TSE diseases in various animals is caused by a prion with very similar characteristics ie BSE and quite distinct from previously recognized scrapie prion strains. |  | | Many of these sites are innervated and eventually the prion gains access to a nerve and then propagates back up the axon to the spinal cord and eventually the brain. |  | | This indicates another compartment in addition to the brain and the LRS must express PrP, if a peripheral prion challenge is to be successful. |
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http://www-micro.msb.le.ac.uk/3035/prions.html
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| | Prion disease definition - Medical Dictionary definitions of popular medical terms |
 | | Fatal familial insomnia (FFI) -- the natural hosts are humans; the prion involved is the FFI prion. |  | | Kuru -- the natural hosts are humans; the prion involved is the kuru prion. |  | | Gerstmann-Sträussler-Scheinker syndrome (GSS) -- the natural hosts are humans; the prion involved is the GSS prion. |
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http://www.medterms.com/script/main/art.asp?articlekey=25932
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| | Science--Pruisner |
 | | As our understanding of prion propagation increases, it should be possible to design effective therapeutics. |  | | prions may have been passed to humans, resulting in a new form |  | | During the past two decades, a previously unknown mechanism of disease has been described in humans and animals. |
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http://www.sciencemag.org/feature/data/prusiner/245.dtl
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| | Introduction to TSEs General Hypothesis |
 | | This hypothesis is based on several worldwide Environmental studies with lab analysis; direct research in cell culture; epidemiological analysis and extensive review of a very wide Scientific literature. |  | | The official theories are very vague and I am not aware of one clearly stated and referenced exposition. |  | | Homeostatic mechanisms, determined by genotype, would try to even out metal imbalance, but are only partially effective in extreme locations and vary from animal to animal.The higher the Mn levels the greater the take up by prions. |
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http://www.purdeyenvironment.com/IntroHyp.htm
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| | Kuru: The Dynamics of a Prion Disease |
 | | Understanding the process of amyloid formation may aid in the development of therapies for such diseases. |  | | A large amount of the work done in the name of understanding prion diseases was carried out by anthropologists in the field studying the Fore. |  | | This is encouraging: if further studies show the PrP gene to be inessential, then physicians may be able to inject antigene therapies to patients with prion diseases in the future (Prusiner, 1995). |
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http://www.as.ua.edu/ant/bindon/ant570/Papers/McGrath/McGrath.htm
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| | Prion Diseases |
 | | had been treated in a new way that no longer destroyed the infectiousness of the scrapie prions. |  | | These interact with each other to form aggregates. |  | | Injections of ground-up brain tissue from an animal or human patient with a prion disease into another animal (of the appropriate species) transmits the disease. |
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http://users.rcn.com/jkimball.ma.ultranet/BiologyPages/P/Prions.html
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| | Clinical Trial: PRION-1: Quinacrine for Human Prion Disease |
 | | It also aims to establish an appropriate framework for the clinical assessment of therapeutic options for human prion disease that can be refined or expanded in the future, as new agents become available. |  | | The human prion diseases have been traditionally classified into Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) disease and kuru. |  | | There is only very limited evidence from laboratory tests for the potential use of quinacrine in human prion disease, and the evidence to date for any possible clinical benefit is very scarce. |
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http://www.clinicaltrials.gov/ct/gui/show/NCT00104663?order=2
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| | Prion finding offers insight into spontaneous protein diseases |
 | | "Our findings gives us the opportunity to start exploring prions on a new level," says Legname. |  | | The researchers believe that their work may also help advance investigations of more common neurodegenerative diseases, such as Alzheimer's disease, Parkinson's disease and amyotrophic lateral sclerosis. |  | | The goal has been to create a bonafide prion in the lab, which would be proven to be such by its ability to infect animals and cause a fatal illness. |
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http://www.eurekalert.org/pub_releases/2004-07/uoc--pfo072704.php
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| | Prion Protein |
 | | This discovery is helping researchers get closer to developing treatments for those with the fatal ailments. |  | | Quinacrine, one investigative treatment for Creutzfeldt-Jakob disease, will soon be tested in a human clinical trial. |  | | The Swiss-cheese holes depicted in this sample of brain tissue from a patient with Creutzfeldt-Jakob disease are a tell-tale sign of spongiform encephalopathy. |
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http://web.sfn.org/content/Publications/BrainBriefings/prion_protein.html
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| | New Scientist Breaking News - Synthesis marks prion disease breakthrough |
 | | "This is an exciting and fascinating initial experiment," says Byron Caughey, a prion researcher at the Rocky Mountain Laboratories in Hamilton, Montana. |  | | According to Prusiner's protein-only prion theory, BSE and related diseases occur when a protein called PrP found in healthy brains assumes a corrupted "prion" shape. |  | | Furthermore, being able to study prions in isolation would give new insights into how PrP folds into a prion and how to prevent or reverse the process to treat prion diseases. |
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http://www.newscientist.com/article.ns?id=dn6219
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| | Prion - Wikipedia, the free encyclopedia |
 | | These diseases affect the structure of brain tissue and all are fatal and untreatable. |  | | Proteins showing prion behaviour are also found in some fungi. |  | | Prion-like proteins that behave in a similar way to PrP are found naturally in some fungi and non-mammalian animals. |
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http://en.wikipedia.org/wiki/Prion
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| | prion - Columbia Encyclopedia® article about prion |
 | | There is no effective treatment for any prion disease. |  | | Normal prion proteins occur naturally in brain tissue. |  | | The prion theory has been controversial from the beginning, and although scientific evidence for the existence of such infectious particles has increased, an exact causal link between prions and the diseases they are believed to cause remains to be established. |
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http://columbia.thefreedictionary.com/prion
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| | Prion disease - Genetics Home Reference |
 | | Prion diseases are a group of progressive conditions that affect the brain and nervous system of humans and animals. |  | | Prion diseases of humans include classic Creutzfeldt-Jakob disease, new variant Creutzfeldt-Jakob disease (a human disorder related to mad cow disease), Gerstmann-Sträussler-Scheinker syndrome, fatal insomnia, and kuru. |  | | Rarely, prion diseases also can be transmitted by exposure to prion-contaminated tissues or other biological materials obtained from individuals with prion disease. |
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http://ghr.nlm.nih.gov/condition=priondisease
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| | prion - Hutchinson encyclopedia article about prion |
 | | It has been suggested that prions consisting of other, non-PrP proteins might be responsible for other degenerative diseases in humans, including Alzheimer's disease. |  | | Prion diseases have been found to arise both by transmission from other individuals (through, for example, diet or medical procedures) and by the inheritance of a mutation in the PrP gene. |  | | Because the immune system does not recognize the prions as foreign, no immune reaction develops. |
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http://encyclopedia.farlex.com/prion
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| | Spiroplasmas as Cause of TSEs (Mad Cow Disease, BSE and CJD). |
 | | A shortcoming in the prion theory is that CJD and scrapie can be transmitted without prions. |  | | Moreover, the prion has been found in unrelated disease processes, such as Kawsaski syndrome and inclusion body myositis. |  | | Some researchers believe prions are the cause of CJD and related illnesses because they have found prions in brain tissue from people with CJD and sheep with scrapie but not in normal brain tissue. |
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http://nov55.com/spr.html
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| | US FDA/CFSAN Bad Bug Book - Prions and Transmissible Spongiform Encephalopathies |
 | | While the "prion theory" of Transmissible Spongiform Encephalopathies (TSEs) is widely accepted, there are other theories of the cause of these illnesses. |  | | The abnormally shaped prions are resistant to most heat and chemical treatments, however certain food manufacturing processes (e.g. |  | | Prions are associated with a group of diseases called Transmissible Spongiform Encephalopathies (TSEs). |
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http://vm.cfsan.fda.gov/~mow/prion.html
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| | HHMI News: “Promiscuous Prion” Yields Clues to Infection Across Species Barriers |
 | | The scientists conducted their studies using yeast prions, which are similar to the mammalian prions that have gained notoriety for their roles in such fatal brain-destroying human diseases such as Creutzfeldt-Jakob disease and kuru, and in the animal diseases BSE and scrapie. |  | | "The key factor will be a prion's strain conformation, which means that it will be critically important to understand on a molecular level the differences among mammalian prion strains." Also, he said, the effects of treatment of prion-containing meat might have enhanced the virulence of the prions. |  | | Weissman and graduate student Peter Chien of UCSF published their studies in the March 8, 2001, issue of the journal Nature. |
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http://www.hhmi.org/news/weissman.html
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| | ScienceDaily: Widespread Cannibalism May Have Caused Prehistoric Prion Disease Epidemics, Science Study Suggests |
 | | Transmissible spongiform encephalopathy -- Transmissible spongiform encephalopathies (TSEs, also known as prion diseases) are a group of progressive conditions that affect the brain and nervous system of humans and animals and are transmitted... |  | | Lab Experiments Reveal Potential Therapeutic Target For Degenerative Brain Diseases (August 3, 1999) -- Laboratory experiments on prion diseases - degenerative brain illnesses such as Kuru and Crutzfeldt-Jakob disease in humans, scrapie in sheep, and the so-called "mad cow disease" - have yielded a... |  | | Prion diseases are caused by misfolded versions of the prion protein, which cause other prion proteins to misfold and clump together in the brain. |
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http://www.sciencedaily.com/releases/2003/04/030411071024.htm
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| | National Prion Disease Pathology Surveillance Center |
 | | The 14-3-3 protein is a marker for some prion diseases, such as Creutzfeldt-Jakob disease (CJD), when a number of other neurodegenerative conditions are excluded. |  | | Acquire tissue samples and clinical information from as many cases of human prion disease occurring in the United States as possible in order to help monitor the possible occurrence of variant CJD (vCJD) in the USA. |  | | Transfer the data obtained to the Centers for Disease Control and Prevention (CDC) and the Health Departments of the individual states in order to monitor the prevalence of prion diseases in the USA and investigate possible cases in which the disease has been acquired from other humans or from animals. |
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http://www.cjdsurveillance.com
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| | Homepage CDC Prion Diseases |
 | | Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. |  | | Prion diseases are usually rapidly progressive and always fatal. |  | | They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. |
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http://www.cdc.gov/ncidod/dvrd/prions
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| | How Prions Work |
 | | Prions main area of attack is the brain. |  | | Once the rogue prions make it, there they begin to change normal proteins until a plaque is formed in the brain. |  | | When a prion causes an infection it causes other similar proteins to change their normal conformations to that of the abnormal form. |
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http://www.rit.edu/~sbib350/posters/prionwork.htm
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| | Advancing Prion Science: Guidance for the National Prion Research Program - Institute of Medicine |
 | | To develop antemortem diagnostics or therapies for TSEs, the committee concludes, NPRP should invest in basic research specifically to elucidate the structural features of prions, the molecular mechanisms of prion replication, the mechanisms of TSE pathogenesis, and the physiological function of prions' normal cellular isoform. |  | | Advancing Prion Science provides the first comprehensive reference on present knowledge about all aspects of TSEs--from basic science to the U.S. research infrastructure; from diagnostics to surveillance; and from prevention to treatment. |  | | Advancing Prion Science: Guidance for the National Prion Research Program - Institute of Medicine |
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http://www.iom.edu/report.asp?id=16514
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| | Mad Cow Disease pictures, prions, BSE, nvCJD images by Russell Kightley Media |
 | | Prions are pathogenic variants of proteins that are naturally produced in nerve cells and certain other cells. |  | | This probably happens by a retrograde flow of prion filled vesicles. |  | | The word "prion" stands for "proteinaceous infectious particle" and so should properly only be applied to the pathogenic variants. |
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http://www.rkm.com.au/BSE
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| | Prion Protein Processing |
 | | In short, a protease-resistant central core of the prion protein ranging in size from 27 to 30kDa is found in amyloid plaques of GSS patients and vCJD patients (although rare in BSE itself) and 75% of Kuru cases, but such amyloid plaques are only found in 5-10% of other CJD patients. |  | | The human prion protein and its normal processing |  | | The processing of the PrP protein is shown graphically below with many of the important landmarks indicated. |
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http://w3.aces.uiuc.edu/AnSci/BSE/Prion_protein_processing.htm
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| | Prion Diseases Research Program, Congressionally Directed Medical Research Programs |
 | | Prion Diseases Research Program, Congressionally Directed Medical Research Programs |  | | The diseases are relatively rare in humans but have been documented most extensively in hoofed mammals. |  | | The current disease theory attributes TSEs to "prions," normal cell membrane proteins with atypical three-dimensional configurations, transmitted by ingestion or possibly blood transfer. |
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http://cdmrp.army.mil/nprp
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| | Pfam 19.0 : Prion |
 | | Prion protein (PrP-c) PUBMED:2572197, PUBMED:1916104, PUBMED:2908696 is a small glycoprotein found in high quantity in the brain of animals infected with certain degenerative neurological diseases, such as sheep scrapie and bovine spongiform encephalopathy (BSE), and the human dementias Creutzfeldt-Jacob disease (CJD) and Gerstmann-Straussler syndrome (GSS). |  | | NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231). |  | | It is thought that the prion protein can exist in two different forms: one is the normal cellular protein, and the other is the infectious form which can change the normal prion protein into the infectious form. |
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http://pfam.wustl.edu/cgi-bin/getdesc?name=Prion
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| | Prion (bird) - Wikipedia, the free encyclopedia |
 | | Antarctic Prion homes on their life mate through smell |  | | For the infectious agent bearing the same name, see prion. |  | | The Antarctic Prion and the Salvin's Prion are usually considered to be races of the Broad-billed Prion. |
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http://en.wikipedia.org/wiki/Prion_(bird)
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| | Turns of Phrase: Prion |
 | | His theory, developed over the past 15 years, is that the infectious agent is not a virus but a protein, which can force other proteins in the infected body to change shape and so cause disease to develop. |  | | The theory, unusually for one gaining a Nobel Prize, is still controversial, with its truth yet to be finally demonstrated experimentally. |  | | prions, an invented word which is a contraction of the full descriptive name proteinaceous infectious particle. |
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http://www.worldwidewords.org/turnsofphrase/tp-pri1.htm
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| | Prion ThirtyTwo, Advanced Snowboard Footwear |
 | | High in value without sacrificing performance and comfort, the Prion is an entry-level boot that can be ridden by a first-timer or a professional. |
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http://www.thirtytwo.com/boots/prion
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| | Prion @CPRMap - Clinical Proteomics Research Map |
 | | Effective early diagnosis of prion diseases in blood |  | | Test could improve detection of prion disease in humans |  | | Tracking movement of the prion protein within brain cells |
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http://www.cprmap.com/forums/forumdisplay.php?f=229
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| | Official Mad Cow Disease Home Page |
 | | 7,431+ articles on mad cow disease, Creutzfeldt-Jacob Disease, prions, spongiform encephalopathies, scrapie, BSE, CJD, CWD, TME, and TSE |  | | 7,651+ articles on mad cow disease, Creutzfeldt-Jakob Disease, VCJD, nvCJD, prions, spongiform encephalopathies, scrapie, BSE, CJD, CWD, TME, and TSE |  | | 7,651+ articles on mad cow and new variantCreutzfeldt-Jakob disease, prions, |
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http://www.mad-cow.org
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| | MSN Encarta - Dictionary - prion (2) definition |
 | | MSN Encarta - Dictionary - prion (2) definition |  | | Click here to search all of MSN Encarta |  | | Search for "prion" in all of MSN Encarta |
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http://encarta.msn.com/encnet/features/dictionary/DictionaryResults.aspx?refid=1861736882
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