| aerosol and nasal transmission of chronic wasting disease in cervidized mice. | little is known regarding the potential risk posed by aerosolized prions. chronic wasting disease (cwd) is transmitted horizontally, almost surely by mucosal exposure, and cwd prions are present in saliva and urine of infected animals. however, whether cwd may be transmissible by the aerosol or nasal route is not known. to address this question, fvb mice transgenetically expressing the normal cervid prp(c) protein [tg(cerprp) mice] were exposed to cwd prions by either nose-only aerosol exposure ... | 2010 | 20164261 |
| prp conformational transitions alter species preference of a prp-specific antibody. | the epitope of the 3f4 antibody most commonly used in human prion disease diagnosis is believed to consist of residues met-lys-his-met (mkhm) corresponding to human prp-(109-112). this assumption is based mainly on the observation that 3f4 reacts with human and hamster prp but not with prp from mouse, sheep, and cervids, in which met at residue 112 is replaced by val. here we report that, by brain histoblotting, 3f4 did not react with prp of uninfected transgenic mice expressing elk prp; however ... | 2010 | 20194495 |
| b cells and platelets harbor prion infectivity in the blood of deer infected with chronic wasting disease. | substantial evidence for prion transmission via blood transfusion exists for many transmissible spongiform encephalopathy (tse) diseases. determining which cell phenotype(s) is responsible for trafficking infectivity has important implications for our understanding of the dissemination of prions, as well as their detection and elimination from blood products. we used bioassay studies of native white-tailed deer and transgenic cervidized mice to determine (i) if chronic wasting disease (cwd) bloo ... | 2010 | 20219916 |
| a novel method for preclinical detection of prpsc in blood. | in this study, we demonstrate that a moderate amount of protein misfolding cyclic amplification (pmca) coupled to a novel surround optical fibre immunoassay (sofia) detection scheme can be used to detect the disease-associated form of the prion protein (prp(sc)) in protease-untreated plasma from preclinical and clinical scrapie sheep, and white-tailed deer with chronic wasting disease, following natural and experimental infection. prp(sc), resulting from a conformational change of the normal (ce ... | 2010 | 20357038 |
| increased risk of chronic wasting disease in rocky mountain elk associated with decreased magnesium and increased manganese in brain tissue. | chronic wasting disease (cwd) is a transmissible spongiform encephalopathy (tse) of rocky mountain elk in north america. recent studies suggest that tissue and blood mineral levels may be valuable in assessing tse infection in sheep and cattle. the objectives of this study were to examine baseline levels of copper, manganese, magnesium, zinc, selenium, and molybdenum in the brains of rocky mountain elk with differing prion genotypes and to assess the association of mineral levels with cwd infect ... | 2010 | 20357959 |
| detection of the abnormal isoform of the prion protein associated with chronic wasting disease in the optic pathways of the brain and retina of rocky mountain elk (cervus elaphus nelsoni). | eyes and nuclei of the visual pathways in the brain were examined in 30 rocky mountain elk (cervus elaphus nelsoni) representing 3 genotypes of the prion protein gene prnp (codon 132: mm, ml, or ll). tissues were examined for the presence of the abnormal isoform of the prion protein associated with chronic wasting disease (prp(cwd)). nuclei and axonal tracts from a single section of brain stem at the level of the dorsal motor nucleus of the vagus nerve were scored for intensity and distribution ... | 2010 | 20382822 |
| pathogenesis of chronic wasting disease in cervidized transgenic mice. | chronic wasting disease (cwd) is a fatal, endemic prion disease of wild and captive cervids, including deer, elk, and moose. typical of prion diseases, cwd is characterized by the conversion of the native, protease-sensitive protein prp(c) to a protease-resistant isoform, denoted as prp(res). here we have studied the expression of cervid prp(c) and the pathogenesis of cwd infection in transgenic mice expressing the normal cervid prion protein (tg[cerprp] mice). using tissue-based in situ immunoh ... | 2010 | 20395435 |
| the role of geographic information systems in wildlife landscape epidemiology: models of chronic wasting disease in colorado mule deer. | the authors present findings from two landscape epidemiology studies of chronic wasting disease (cwd) in northern colorado mule deer (odocoileus hemionus). first, the effects of human land use on disease prevalence were explored by formulating a set of models estimating cwd prevalence in relation to differences in human land use, sex and geographic location. prevalence was higher in developed areas and among male deer suggesting that anthropogenic influences (changes in land use), differences in ... | 2007 | 20422537 |
| iron content of ferritin modulates its uptake by intestinal epithelium: implications for co-transport of prions. | the spread of chronic wasting disease (cwd) in the deer and elk population has caused serious public health concerns due to its potential to infect farm animals and humans. like other prion disorders such a sporadic creutzfeldt-jakob-disease of humans and mad cow disease of cattle, cwd is caused by prp-scrapie (prpsc), a beta-sheet rich isoform of a normal cell surface glycoprotein, the prion protein (prpc). since prpsc is sufficient to cause infection and neurotoxicity if ingested by a suscepti ... | 2010 | 20429907 |
| experimental oral transmission of chronic wasting disease to red deer (cervus elaphus elaphus): early detection and late stage distribution of protease-resistant prion protein. | chronic wasting disease (cwd), an important emerging prion disease of cervids, is readily transmitted by intracerebral or oral inoculation from deer-to-deer and elk-to-elk, suggesting the latter is a natural route of exposure. studies of host range susceptibility to oral infection, particularly of those species found in habitats where cwd currently exists are imperative. this report describes the experimental transmission of cwd to red deer following oral inoculation with infectious cwd material ... | 2010 | 20436863 |
| enzymatic digestion of chronic wasting disease prions bound to soil. | chronic wasting disease (cwd) and sheep scrapie can be transmitted via indirect environmental routes, and it is known that soil can serve as a reservoir of prion infectivity. given the strong interaction between the prion protein (prp) and soil, we hypothesized that binding to soil enhances prion resistance to enzymatic digestion, thereby facilitating prion longevity in the environment and providing protection from host degradation. we characterized the performance of a commercially available su ... | 2010 | 20450190 |
| prion strain mutation determined by prion protein conformational compatibility and primary structure. | prions are infectious proteins composed of the abnormal disease-causing isoform prpsc, which induces conformational conversion of the host-encoded normal cellular prion protein prpc to additional prpsc. the mechanism underlying prion strain mutation in the absence of nucleic acids remains unresolved. additionally, the frequency of strains causing chronic wasting disease (cwd), a burgeoning prion epidemic of cervids, is unknown. using susceptible transgenic mice, we identified two prevalent cwd s ... | 2010 | 20466881 |
| medicine. prion strain mutation and selection. | | 2010 | 20508117 |
| faecal cwd prion excretion and inflammation. | chronic wasting disease (cwd) is a transmissible spongiform encephalopathy (tse)--or prion disease (pd)--that has become of increasing concern throughout years among different captive and free-living deer species and populations in north america. starting from the recent pre-clinical evidence of prion infectivity in faecal material from cwd-infected mule deer (odocoileus hemionus), this contribution takes into special consideration the potential role of certain gut inflammatory conditions as a f ... | 2010 | 20518283 |
| cell-based quantification of chronic wasting disease prions. | cell-based measurement of prion infectivity is currently restricted to experimental strains of mouse-adapted scrapie. having isolated cell cultures with susceptibility to prions from diseased elk, we describe a modification of the scrapie cell assay allowing evaluation of prions causing chronic wasting disease, a naturally occurring transmissible spongiform encephalopathy. we compare this cervid prion cell assay to bioassays in transgenic mice, the only other existing method for quantification, ... | 2010 | 20519392 |
| sensitive detection of scrapie prion protein in soil. | prion diseases are fatal neurodegenerative disorders that are caused by infectious agents known as prions. prions are composed primarily of the pathogenic prion protein isoform, prp(sc). because significant levels of infectivity have been detected in excrement from animals infected with scrapie and chronic wasting disease, studies on the dynamics of prp(sc) levels in contaminated soil are needed to assess the possible horizontal transmission of prion diseases. using protein misfolding cyclic amp ... | 2010 | 20570651 |
| molecular genealogy tools for white-tailed deer with chronic wasting disease. | molecular genetic data provide powerful tools for genealogy reconstruction to reveal mechanisms underlying disease ecology. white-tailed deer (odocoileus virginianus) congregate in matriarchal groups; kin-related close social spacing may be a factor in the spread of infectious diseases. spread of chronic wasting disease (cwd), a prion disorder of deer and their cervid relatives, is presumed to be associated with direct contact between individuals and by exposure to shared food and water sources ... | 2010 | 20592847 |
| prion interaction with the 37-kda/67-kda laminin receptor on enterocytes as a cellular model for intestinal uptake of prions. | enterocytes, a major cell population of the intestinal epithelium, represent one possible barrier to the entry of prions after oral exposure. we established a cell culture system employing enterocytes from different species to study alimentary prion interaction with the 37-kda/67-kda laminin receptor lrp/lr. human, bovine, porcine, ovine, and cervid enterocytes were cocultured with brain homogenates from cervid, sheep, and cattle suffering from chronic wasting disease (cwd), scrapie, and bovine ... | 2010 | 20603132 |
| chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. | chronic wasting disease (cwd) is a prion disease that affects free-ranging and captive cervids, including mule deer, white-tailed deer, rocky mountain elk and moose. cwd-infected cervids have been reported in 14 usa states, two canadian provinces and in south korea. the possibility of a zoonotic transmission of cwd prions via diet is of particular concern in north america where hunting of cervids is a popular sport. to investigate the potential public health risks posed by cwd prions, we have in ... | 2010 | 20610667 |
| immunotherapy for prion diseases: opportunities and obstacles. | transmissible spongiform encephalopathies (tses) represent a unique form of infectious disease based on the misfolding of a self-protein into a pathological conformation. while other human diseases are also attributed to protein misfolding, the tses are unique in their zoonotic potential and iatrogenic infectivity. these characteristics are of particular importance in the aftermath of the uk bovine spongiform encephalopathy (bse) outbreak due to the dual concerns that a subpopulation of individu ... | 2010 | 20635933 |
| select tissue mineral concentrations and chronic wasting disease status in mule deer from north-central colorado. | trace mineral imbalances have been suggested as having a causative or contributory role in chronic wasting disease (cwd), a prion disease of several north american cervid species. to begin exploring relationships between tissue mineral concentrations and cwd in natural systems, we measured liver tissue concentrations of copper, manganese, and molybdenum in samples from 447 apparently healthy, adult (> or = 2 yr old) mule deer (odocoileus hemionus) culled or vehicle killed from free-ranging popul ... | 2010 | 20688718 |
| causes of mortality and diseases in farmed deer in switzerland. | to investigate diseases and causes of mortality in swiss farmed deer, deer found dead or shot due to diseased condition between march 2003 and december 2004 were requested for a complete postmortem examination. one hundred and sixty-two animals were submitted. perinatal mortality, necrobacillosis in 3 week to 6 month old deer, and endoparasitosis in 6 month to 2 year old deer were identified as the most important causes of loss, followed by ruminal acidosis, which was diagnosed in 22% of deer ol ... | 2010 | 20706668 |
| environmental sources of scrapie prions. | ovine scrapie and cervine chronic wasting disease show considerable horizontal transmission. here we report that a scrapie-affected sheep farm has a widespread environmental contamination with prions. prions were amplified by protein-misfolding cyclic amplification (spmca) from seven of nine environmental swab samples taken, including those from metal, plastic, and wooden surfaces. sheep had been removed from the areas from which the swabs were taken up to 20 days prior to sampling, indicating t ... | 2010 | 20739536 |
| transport of the pathogenic prion protein through soils. | transmissible spongiform encephalopathies (tses) are progressive neurodegenerative diseases and include bovine spongiform encephalopathy of cattle, chronic wasting disease (cwd) of deer and elk, scrapie in sheep and goats, and creutzfeldt-jakob disease in humans. an abnormally folded form of the prion protein (designated prp(tse)) is typically associated with tse infectivity and may constitute the major, if not sole, component of the infectious agent. transmission of cwd and scrapie is mediated ... | 2010 | 20830901 |
| estimating chronic wasting disease effects on mule deer recruitment and population growth. | chronic wasting disease (cwd), a prion disease of mule deer (odocoileus hemionus), accelerates mortality and in so doing has the potential to influence population dynamics. although effects on mule deer survival are clear, how cwd affects recruitment is less certain. we studied how prion infection influenced the number of offspring raised to weaning per adult (≥2 yr old) female mule deer and subsequently the estimated growth rate (λ) of an infected deer herd. infected and presumably uninfected r ... | 2010 | 20966260 |
| the interaction of ruminant prp(sc) with soils is influenced by prion source and soil type. | the persistence of prions within the environment is implicated in the horizontal transmission of ovine scrapie and cervid chronic wasting disease. description of the interaction of prion strains derived from their natural hosts with a range of soil types is imperative in understanding how prions persist in the environment and, therefore, the characteristics of prion transmission. here, we demonstrate that all detectable ovine scrapie and bovine bse prp(sc) bind to a range of soil types within 24 ... | 2010 | 20968294 |
| cwd prevalence, perceived human health risks, and state influences on deer hunting participation. | this study examined factors predicted by previous research to influence hunters' decisions to stop hunting deer in a state. data were obtained from mail surveys of resident and nonresident deer hunters in arizona, north dakota, south dakota, and wisconsin (n = 3,518). hunters were presented with six scenarios depicting hypothetical cwd prevalence levels and human health risks from the disease (e.g., death), and asked if they would continue or stop hunting deer in the state. bivariate analyses ex ... | 2010 | 21039704 |
| minor oral lesions facilitate transmission of chronic wasting disease. | while chronic wasting disease (cwd) prion transmission, entry, and trafficking remain incompletely elucidated, natural exposure of the oral and/or nasal mucous membranes seems certain. cervids commonly sustain minor lesions on oral mucous membranes that could have an impact on susceptibility to prion infection. to explore this potential cofactor, we studied cohorts of cervid prp transgenic mice with or without superficial abrasions on the lingual mucosa to determine whether minor oral mucosa les ... | 2010 | 21084472 |
| association analysis of prnp gene region with chronic wasting disease in rocky mountain elk. | abstract: | 2010 | 21087518 |
| immunomodulation for prion and prion-related diseases. | prion diseases are a unique category of illness, affecting both animals and humans, where the underlying pathogenesis is related to a conformational change of a normal self protein called cellular prion protein to a pathological and infectious conformer known as scrapie prion protein (prp(sc)). currently, all prion diseases lack effective treatment and are universally fatal. past experiences with bovine spongiform encephalopathy and variant creutzfeldt-jakob disease mainly in europe, as well as ... | 2010 | 21105779 |
| monitoring immune cells trafficking fluorescent prion rods hours after intraperitoneal infection. | presence of an abnormal form a host-encoded prion protein (prpc) that is protease resistant, pathologic and infectious characterizes prion diseases such as chronic wasting disease (cwd) of cervids and scrapie in sheep. the prion hypothesis asserts that this abnormal conformer constitutes most or all of the infectious prion. the role of the immune system in early events in peripheral prion pathogenesis has been convincingly demonstrated for cwd and scrapie. transgenic and pharmacologic studies in ... | 2010 | 21113122 |
| alimentary prion infections: touchdown in the intestine. | neurodegenerative diseases are caused by proteinaceous aggregates, usually consisting of misfolded proteins which are often typified by a high proportion of β-sheets, which accumulate in the central nervous system. these diseases, including morbus alzheimer, parkinson disease and transmissible spongiform encephalopathies (tses)--also termed prion disorders--afflict a substantial proportion of the human population and as such the etiology and pathogenesis of these diseases has been the focus of m ... | 2011 | 21150306 |
| rapid end-point quantitation of prion seeding activity with sensitivity comparable to bioassays. | a major problem for the effective diagnosis and management of prion diseases is the lack of rapid high-throughput assays to measure low levels of prions. such measurements have typically required prolonged bioassays in animals. highly sensitive, but generally non-quantitative, prion detection methods have been developed based on prions' ability to seed the conversion of normally soluble protease-sensitive forms of prion protein to protease-resistant and/or amyloid fibrillar forms. here we descri ... | 2010 | 21152012 |
| homogenization of large-scale movement models in ecology. | a difficulty in using diffusion models to predict large scale animal population dispersal is that individuals move differently based on local information (as opposed to gradients) in differing habitat types. this can be accommodated by using ecological diffusion. however, real environments are often spatially complex, limiting application of a direct approach. homogenization for partial differential equations has long been applied to fickian diffusion (in which average individual movement is org ... | 2011 | 21194012 |
| spiroplasma found in the eyes of scrapie affected sheep. | scrapie, a transmissible spongiform encephalopathy (tse) occurring naturally in sheep, characteristically shows a severe retinopathy that is well developed in the terminal phases of the disease. in this study, we set out to demonstrate similar retinal changes in our ruminant spiroplasmosis tse model. | 2011 | 21199275 |
| generation of a new form of human prp(sc) in vitro by interspecies transmission from cervid prions. | prion diseases are infectious neurodegenerative disorders that affect humans and animals and that result from the conversion of normal prion protein (prp(c)) into the misfolded prion protein (prp(sc)). chronic wasting disease (cwd) is a prion disorder of increasing prevalence within the united states that affects a large population of wild and captive deer and elk. determining the risk of transmission of cwd to humans is of utmost importance, considering that people can be infected by animal pri ... | 2011 | 21209079 |
| prionet canada: a network of centres of excellence for research on prion diseases--ongoing and future research directions. | it is prionet's vision to build a network that shapes and sustains prion research in canada, translating basic science into accessible socioeconomic benefits for global betterment. prionet's research is developing surveillance measures, diagnostic tools, vaccines, and potential therapies and determining the various impacts of prion diseases on people. prionet seeks to integrate scientifically informed risk management strategies and to use this knowledge to address ongoing problems posed by bovin ... | 2011 | 21218336 |
| the role of predation in disease control: a comparison of selective and nonselective removal on prion disease dynamics in deer. | effective measures for controlling chronic wasting disease (cwd), a contagious prion disease of cervids, remain elusive. we review theoretic relationships between predation and host-parasite dynamics and describe a mathematical model to evaluate the potential influence of random removal through harvest or culling and selective predation by wolves (canis lupus) upon cwd dynamics in deer (odocoileus spp.) populations. imposing nonselective mortality representing a 15% annual harvest or cull 51 yr ... | 2011 | 21269999 |
| sparse prp(sc) accumulation in the placentas of goats with naturally acquired scrapie. | domestic goats (capra hircus) are a natural and experimental host of scrapie and bovine spongiform encephalopathy, the transmissible spongiform encephalopathies (tse) of sheep and cattle. goats are also susceptible to experimental infection with the agents of tses of deer and elk (chronic wasting disease) and humans (creutzfeldt jakob disease). distribution of prpsc, the abnormal prion protein, is similar in the tissues of scrapie-infected sheep and goats but no data are available on the potenti ... | 2011 | 21284878 |
| living dangerously with big fancy models. | | 2010 | 21302819 |
| advances in modeling highlight a tension between analytical accuracy and accessibility. | | 2010 | 21302820 |
| model complexity and information in the data: could it be a house built on sand? | | 2010 | 21302821 |
| are exercises like this a good use of anybody's time? | | 2010 | 21302822 |
| atomic structures suggest determinants of transmission barriers in mammalian prion disease. | prion represents a unique class of pathogens devoid of nucleic acid. the deadly diseases transmitted by it between members of one species and, in certain instances, to members of other species present a public health concern. transmissibility and the barriers to transmission between species have been suggested to arise from the degree to which a pathological protein conformation from an individual of one species can seed a pathological conformation in another species. however, this hypothesis ha ... | 2011 | 21323366 |
| biological effects and use of prpsc- and prp-specific antibodies generated by immunization with purified full-length native mouse prions. | the prion agent is the infectious particle causing spongiform encephalopathies in animals and humans and is thought to consist of an altered conformation (prp(sc)) of the normal and ubiquitous prion protein prp(c). the interaction of the prion agent with the immune system, particularly the humoral immune response, has remained unresolved. here we investigated the immunogenicity of full-length native and infectious prions, as well as the specific biological effects of the resulting monoclonal ant ... | 2011 | 21345946 |
| replication efficiency of soil-bound prions varies with soil type. | prion sorption to soil is thought to play an important role in the transmission of scrapie and chronic wasting disease (cwd) via the environment. sorption of prp to soil and soil minerals is influenced by the strain and species of prp(sc) and by soil characteristics. however, the ability of soil-bound prions to convert prp(c) to prp(sc) under these wide-ranging conditions remains poorly understood. we developed a semiquantitative protein misfolding cyclic amplification (pmca) protocol to evaluat ... | 2011 | 21430062 |
| prion protein polymorphisms affect chronic wasting disease progression. | analysis of the prnp gene in cervids naturally infected with chronic wasting disease (cwd) suggested that prnp polymorphisms affect the susceptibility of deer to infection. to test this effect, we orally inoculated 12 white-tailed deer with cwd agent. three different prnp alleles, wild-type (wt; glutamine at amino acid 95 and glycine at 96), q95h (glutamine to histidine at amino acid position 95) and g96s (glycine to serine at position 96) were represented in the study cohort with 5 wt/wt, 3 wt/ ... | 2011 | 21445256 |
| investigation of the effects of experimental autolysis on the detection of abnormal prion protein in lymphoid and central nervous system tissues from elk and sheep using the western blotting method. | tissues unsuitable for standard immunohistochemical and histopathological examinations for chronic wasting disease (cwd) in cervids and for scrapie in sheep are frequently submitted for testing. this study investigated the effects of experimental autolysis on the detection of abnormal prion protein (prpsc) in lymphoid and central nervous system (cns) tissues from elk and sheep. the prpsc was detected using a western blotting (wb) test following prpsc enrichment using sodium phosphotungstic acid ... | 2011 | 21461199 |
| molecular dynamics studies on the structural stability of wild-type dog prion protein. | prion diseases such as creutzfeldt-jakob disease, variant creutzfeldt-jakob diseases, gerstmann-sträussler-scheinker syndrome, fatal familial insomnia, kuru in humans, scrapie in sheep, bovine spongiform encephalopathy (or 'mad-cow' disease) and chronic wasting disease in cattle are invariably fatal and highly infectious neurodegenerative diseases affecting humans and animals. however, by now there have not been some effective therapeutic approaches to treat all these prion diseases. in 2008, ca ... | 2011 | 21469747 |
| presence and seeding activity of pathological prion protein (prp(tse)) in skeletal muscles of white-tailed deer infected with chronic wasting disease. | chronic wasting disease (cwd) is a contagious, rapidly spreading transmissible spongiform encephalopathy (tse), or prion disease, occurring in cervids such as white tailed-deer (wtd), mule deer or elk in north america. despite efficient horizontal transmission of cwd among cervids natural transmission of the disease to other species has not yet been observed. here, we report for the first time a direct biochemical demonstration of pathological prion protein prp(tse) and of prp(tse)-associated se ... | 2011 | 21483771 |
| fate of prions in soil: a review. | prions are the etiological agents of transmissible spongiform encephalopathies (tsses), a class of fatal neurodegenerative diseases affecting humans and other mammals. the pathogenic prion protein is a misfolded form of the host-encoded prion protein and represents the predominant, if not sole, component of the infectious agent. environmental routes of tse transmission areimplicated in epizootics of sheep scrapie and chronic wasting disease (cwd) of deer, elk, and moose. soil represents a plausi ... | 2011 | 21520752 |
| detection of cwd prions in salivary, urinary, and intestinal tissues of deer: potential mechanisms of prion shedding and transmission. | efficient horizontal transmission is a signature trait of chronic wasting disease (cwd) in cervids. infectious prions shed into excreta appear to play a key role in this facile transmission, as has been demonstrated by bioassay in cervid and transgenic species and serial protein misfolding cyclic amplification (spmca). however, the source(s) of infectious prions in these body fluids have yet to be identified. in the present study, we analyzed tissues proximate to saliva, urine, and feces product ... | 2011 | 21525361 |
| effects of solution chemistry and aging time on prion protein adsorption and replication of soil-bound prions. | prion interactions with soil may play an important role in the transmission of chronic wasting disease (cwd) and scrapie. prions are known to bind to a wide range of soil surfaces, but the effects of adsorption solution chemistry and long-term soil binding on prion fate and transmission risk are unknown. we investigated hy tme prion protein (prp(sc)) adsorption to soil minerals in aqueous solutions of phosphate buffered saline (pbs), sodium chloride, calcium chloride, and deionized water using w ... | 2011 | 21526178 |
| genetics of prion disease. | prion diseases or transmissible spongiform encephalopathies (tses) are neurodegenerative disorders of humans and animals for which there are no effective treatments or cure. they include creutzfeldt-jakob disease (cjd) in humans and sheep scrapie, bovine spongiform encephalopathy (bse) and chronic wasting disease (cwd) in cervids. the prion protein (prp) is central to the disease process. an abnormal form of prp is generally considered to be the sole or principal component of the infectious agen ... | 2011 | 21528440 |
| an enzymatic treatment of soil-bound prions effectively inhibits replication. | chronic wasting disease (cwd) and scrapie can be transmitted through indirect environmental routes, possibly via soil, and a practical decontamination strategy for prion-contaminated soil is currently unavailable. in the laboratory, an enzymatic treatment under environmentally-relevant conditions (22°c, ph 7.4) can degrade soil-bound prp(sc) below the limits of western blot detection. we developed and used a quantitative serial protein misfolding cyclic amplification (pmca) protocol to character ... | 2011 | 21571886 |
| atypical prion diseases in humans and animals. | although prion diseases, such as creutzfeldt-jakob disease (cjd) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. protease-resistant conformers of the prion protein (prp), known as the "scrapie form" (prp(sc)), are used as disease markers, and for taxonomic purposes, in ... | 2011 | 21598097 |
| chronic wasting disease. | chronic wasting disease (cwd) is a prion disease of free-ranging and farmed ungulates (deer, elk, and moose) in north america and south korea. first described by the late e.s. williams and colleagues in northern colorado and southern wyoming in the 1970s, cwd has increased tremendously both in numerical and geographical distribution, reaching prevalence rates as high as 50% in free-ranging and >90% in captive deer herds in certain areas of usa and canada. cwd is certainly the most contagious pri ... | 2011 | 21598099 |
| modeling routes of chronic wasting disease transmission: environmental prion persistence promotes deer population decline and extinction. | chronic wasting disease (cwd) is a fatal disease of deer, elk, and moose transmitted through direct, animal-to-animal contact, and indirectly, via environmental contamination. considerable attention has been paid to modeling direct transmission, but despite the fact that cwd prions can remain infectious in the environment for years, relatively little information exists about the potential effects of indirect transmission on cwd dynamics. in the present study, we use simulation models to demonstr ... | 2011 | 21603638 |
| travel history, hunting, and venison consumption related to prion disease exposure, 2006-2007 foodnet population survey. | the transmission of bovine spongiform encephalopathy (bse) to human beings and the spread of chronic wasting disease (cwd) among cervids have prompted concerns about zoonotic transmission of prion diseases. travel to the united kingdom and other european countries, hunting for deer or elk, and venison consumption could result in the exposure of us residents to the agents that cause bse and cwd. the foodborne diseases active surveillance network 2006-2007 population survey was used to assess the ... | 2011 | 21616198 |
| medical microbiology | subacute progressive degenerative diseases of the nervous system are important
because they appear to be caused by infectious agents that are smaller than
conventional viruses and composed mainly of protein related to a cell protein. few,
if any, of these diseases are curable. although some are genetically determined,
most occur sporadically, and a history of the disease does not appear in close
relatives. therefore, ... | 1996 | 21413288 |
| cytokine antibody array analysis in brain and periphery of scrapie-infected tg338 mice. | scrapie is a prion-associated transmissible spongiform encephalopathy (tse) of sheep and goats, and frequently serves as a comparative model for other prion diseases, such as chronic wasting disease and bovine spongiform encephalopathy. tses are unique neurologic disorders that do not appear to be accompanied by robust systemic immunologic responses. mrna data suggest that cytokines are involved in scrapie progression. in this study, brain tissue, mesenteric lymph nodes, splenic tissue and serum ... | 2011 | 21788075 |
| chronic wasting disease prion trafficking via the autonomic nervous system. | chronic wasting disease (cwd) is a fatal spongiform encephalopathy that is efficiently transmitted among members of the mammalian family cervidae, including deer, elk, and moose. typical of prion diseases, cwd is characterized by the conversion of the native protease-sensitive protein prp(c) to a protease-resistant isoform, denoted prp(res). in native species, spread of the disease likely results from the ingestion of prion-containing excreta, including urine, saliva, or feces. although cervid p ... | 2011 | 21777560 |
| stable limit cycles and the paradox of enrichment in a model of chronic wasting disease. | prions, which cause chronic wasting disease and other transmissible spongiform encephalopathies in ungulates, can remain active in soils for years. the reproductive age of ungulate populations is well within the residence time of prions in the soil. reproduction and mortality in disease-free wildlife populations is regulated by density-dependent mechanisms, which also underlie the concept of carrying capacity. here, we present a model of a susceptible deer population with density-dependent popul ... | 2011 | 21774409 |
| enhancement of immunohistochemical staining of scrapie proteins and immune cells within lymph nodes of early scrapie-infected sheep. | transmissible spongiform encephalopathies (tse) are a group of fatal neurodegenerative diseases that affect animals as well as humans. the oldest of these diseases is scrapie seen in sheep. scrapie is caused by an altered form (prp(sc)), capable of inducing "self-replication" of the normal host prion protein(prp(c)). there is currently no universal standard for antigen retrieval when using immunohistochemistry to simultaneously stain the prp(c) protein and other cellular markers. the use of form ... | 2011 | 21722647 |
| alteration of the chronic wasting disease species barrier by in vitro prion amplification. | chronic wasting disease (cwd) is a transmissible spongiform encephalopathy (tse) of cervids now detected in 19 states of the united states, three canadian provinces, and south korea. whether noncervid species can be infected by cwd and thereby serve as reservoirs for the infection is not known. to investigate this issue, we previously used serial protein misfolding cyclic amplification (spmca) to demonstrate that cwd prions can amplify in brain homogenates from several species sympatric with cer ... | 2011 | 21697475 |
| prion disease detection, pmca kinetics, and igg in urine from sheep naturally/experimentally infected with scrapie and deer with preclinical/clinical chronic wasting disease. | prion diseases, also known as transmissible spongiform encephalopathies, are fatal neurodegenerative disorders. low levels of infectious agent and limited, infrequent success of disease transmissibility and prp(sc) detection have been reported with urine from experimentally infected clinical cervids and rodents. we report the detection of prion disease-associated seeding activity (pasa) in urine from naturally and orally infected sheep with clinical scrapie agent and orally infected preclinical ... | 2011 | 21715495 |
| in vivo comparison of chronic wasting disease infectivity from deer with variation at prion protein residue 96. | chronic wasting disease (cwd) is a prion disease of cervids that causes neurodegeneration and death. susceptibility to prion infections, including cwd, can be dependent on the amino acid sequence of the host prion protein (prp). here, cwd agent obtained from a deer expressing the 96ss genotype, associated with partial resistance to cwd, was used to infect transgenic (tg) mice expressing either 96gg or 96ss deer prp. transgenic mice expressing 96gg deer prp succumbed to this agent, but tg mice ex ... | 2011 | 21697479 |
| using wild white-tailed deer to detect eastern equine encephalitis virus activity in maine. | abstract serum from 226 free-ranging white-tailed deer (odocoileus virginianus) was screened for eastern equine encephalitis virus (eeev) antibodies using plaque reduction neutralization tests. eeev antibodies were detected in 7.1% of samples. this is the first time eeev antibodies have been detected in o. virginianus populations in the state of maine (me). the highest percentage of eeev positive sera was in somerset county (19%) in central me, and this is the first time that eeev activity has b ... | 2011 | 21736489 |
| failure of fallow deer (dama dama) to develop chronic wasting disease when exposed to a contaminated environment and infected mule deer (odocoileus hemionus). | we monitored a herd of fallow deer (dama dama) for evidence of prion infection for 7 yr by periodic postmortem examination of animals from the herd. the fallow deer were exposed to the chronic wasting disease (cwd) agent from mule deer by living in a paddock considered contaminated with infectivity from its history of housing cwd infected deer and, after the first year of the study, by comingling with infected mule deer (odocoileus hemionus). at least 8 of 12 mule deer serving as sentinels for p ... | 2011 | 21719844 |
| real-time quaking-induced conversion: a highly sensitive assay for prion detection. | we recently developed a new in vitro amplification technology, designated "real-time quaking-induced conversion (rt-quic)", for detection of the abnormal form of prion protein (prpsc) in easily accessible specimens such as cerebrospinal fluid (csf). after assessment of more than 200 csf specimens from japanese and australian patients, we found no instance of a false positive, and more than 80% accuracy for the correct diagnosis of sporadic creutzfeldt-jakob disease (scjd). furthermore, the rt-qu ... | 2011 | 21778820 |
| experimental transmission of chronic wasting disease (cwd) from elk and white-tailed deer to fallow deer by intracerebral route: final report. | final observations on experimental transmission of chronic wasting disease (cwd) from elk (cervus elaphus nelsoni) and white-tailed deer (odocoileus virginianus) to fallow deer (dama dama) are reported herein. during the 5-year study, 13 fawns were inoculated intracerebrally with cwd-infected brain material from white-tailed deer (n = 7; group a) or elk (n = 6; group b), and 3 other fawns were kept as uninoculated controls (group c). as described previously, 3 cwd-inoculated deer were euthanized ... | 2011 | 21731188 |
| The economic impacts of chronic wasting disease and bovine spongiform encephalopathy in alberta and the rest of Canada. | Input-output analysis was used to calculate the economic impacts from potential prion diseases outbreaks in Alberta and the rest of Canada. Both chronic wasting disease (CWD) and bovine spongiform encephalopathy (BSE) have the capacity not only to affect the farmed cervid and cattle industries, but also to impact all industries with direct and indirect links to these sectors. Cervid sector shocks yield small spillover effects on the economies of Alberta as well as that of all of Canada. In contr ... | 2011 | 22043917 |
| an nmr metabolomics study of elk inoculated with chronic wasting disease. | chronic wasting disease (cwd) is a fatal neurodegenerative disease affecting both farmed and wild cervids, specifically deer and elk, and is a member of the larger family of prion diseases. prion disease transmission is believed to occur through exposure to infectious prion material-a misfolded and infectious form of the prion protein that is normally present in the host. chronic wasting disease is endemic to regions of central north america and infectious material can persist for long periods i ... | 2011 | 22043909 |
| experimental interspecies transmission studies of the transmissible spongiform encephalopathies to cattle: comparison to bovine spongiform encephalopathy in cattle. | prion diseases or transmissible spongiform encephalopathies (tses) of animals include scrapie of sheep and goats; transmissible mink encephalopathy (tme); chronic wasting disease (cwd) of deer, elk and moose; and bovine spongiform encephalopathy (bse) of cattle. the emergence of bse and its spread to human beings in the form of variant creutzfeldt-jakob disease (vcjd) resulted in interest in susceptibility of cattle to cwd, tme and scrapie. experimental cross-species transmission of tse agents p ... | 2011 | 21908269 |
| White-tailed deer are susceptible to the agent of sheep scrapie by intracerebral inoculation. | ABSTRACT: Interspecies transmission studies afford the opportunity to better understand the potential host range and origins of prion diseases. The purpose of this experiment was to determine susceptibility of white-tailed deer to the agent of scrapie after intracerebral inoculation and to compare clinical signs and lesions to those reported for chronic wasting disease (CWD). Deer (n = 5) were inoculated with 1 mL of a 10% (wt/vol) brain homogenate derived from a sheep clinically affected with s ... | 2011 | 21988781 |
| an overview of animal prion diseases. | prion diseases are transmissible neurodegenerative conditions affecting human and a wide range of animal species. the pathogenesis of prion diseases is associated with the accumulation of aggregates of misfolded conformers of host-encoded cellular prion protein (prpc). animal prion diseases include scrapie of sheep and goats, bovine spongiform encephalopathy (bse) or mad cow disease, transmissible mink encephalopathy, feline spongiform encephalopathy, exotic ungulate spongiform encephalopathy, c ... | 2011 | 22044871 |
| targeting the detection of chronic wasting disease using the hunter harvest during early phases of an outbreak in saskatchewan, canada. | chronic wasting disease (cwd) is a fatal disease of north american cervids that was first detected in a wild, hunter-shot deer in saskatchewan along the border with alberta in canada in 2000. spatially explicit models for assessing factors affecting disease detection are needed to guide surveillance and control programs. spatio-temporal patterns in cwd prevalence can be complicated by variation in individual infection probability and sampling biases. we assessed hunter harvest data of mule deer ... | 2011 | 22137503 |
| the impact of chronic wasting disease and its management on hunter perceptions, opinions, and behaviors in alberta, canada. | the goal of this analysis was to identify changes in hunting behavior, satisfaction, and perceptions of risk in the presence of chronic wasting disease (cwd). hunters completed an internet survey containing direct questions regarding the impacts of cwd and gathering information about real and hypothetical hunting trips. overall, hunters were satisfied with cwd management, and although certain behaviors were altered, the perceived risk by hunters did not seem to be high. a travel cost model was u ... | 2011 | 22043918 |
| prpsc detection in formalin-fixed paraffin-embedded tissue by elisa. | abstract: | 2011 | 22018205 |
| immunophenotype of cells within cervine rectoanal mucosa-associated lymphoid tissue and mesenteric lymph nodes. | rectoanal mucosa-associated lymphoid tissue (ramalt) is a part of the lymphoid system that can be sampled easily in live animals, especially ruminants. ramalt biopsy is useful for the diagnosis of transmissible spongiform encephalopathies, including scrapie in sheep and goats and chronic wasting disease (cwd) in cervids. diagnosis is reliant on detection of abnormal prion protein (prp(d)), which is associated with lymphoid follicles. for enzyme linked immunosorbent assays (elisas) detecting prp( ... | 2011 | 22000034 |
| evidence for distinct chronic wasting disease (cwd) strains in experimental cwd in ferrets. | chronic wasting disease (cwd) is an evolving prion disease of cervids (deer, elk and moose) that has been recognized in north america and korea. infection of non-cervid reservoir or transport species in nature is not reported. however, the ferret (mustela putorius furo) is susceptible to cwd after experimental inoculation. here, we report that infection of ferrets with either of two ferret cwd isolates by various routes of exposure has revealed biologically distinct strain-like properties distin ... | 2012 | 21918005 |
| The structural stability of wild-type horse prion protein. | Prion diseases (e.g. Creutzfeldt-Jakob disease (CJD), variant CJD (vCJD), Gerstmann-Straussler-Scheinker syndrome (GSS), Fatal Familial Insomnia (FFI) and Kuru in humans, scrapie in sheep, bovine spongiform encephalopathy (BSE or 'mad-cow' disease) and chronic wasting disease (CWD) in cattles) are invariably fatal and highly infectious neurodegenerative diseases affecting humans and animals. However, by now there have not been some effective therapeutic approaches or medications to treat all the ... | 2011 | 21875155 |
| transcriptional modulation in a leukocyte-depleted splenic cell population during prion disease. | prion replication in the periphery precedes neuroinvasion in many experimental rodent scrapie models, and in natural sheep scrapie and chronic wasting disease (cwd) in cervids. prions propagate in the germinal centers of secondary lymphoid organs and are strongly associated with follicular dendritic cells (fdc) and possibly circulating dendritic cells and macrophages. given the importance of lymphoid organs in prion disease transmission and pathogenesis, gene expression studies may reveal host f ... | 2011 | 22043911 |
| diversity and distribution of white-tailed deer mtdna lineages in chronic wasting disease (cwd) outbreak areas in southern wisconsin, usa. | chronic wasting disease (cwd) is a transmissible spongiform encephalopathy affecting north american cervids. because it is uniformly fatal, the disease is a major concern in the management of white-tailed deer populations. management programs to control cwd require improved knowledge of deer interaction, movement, and population connectivity that could influence disease transmission and spread. genetic methods were employed to evaluate connectivity among populations in the cwd management zone of ... | 2011 | 22043912 |
| transmission of chronic wasting disease identifies a prion strain causing cachexia and heart infection in hamsters. | chronic wasting disease (cwd) is an emerging prion disease of free-ranging and captive cervids in north america. in this study we established a rodent model for cwd in syrian golden hamsters that resemble key features of the disease in cervids including cachexia and infection of cardiac muscle. following one to three serial passages of cwd from white-tailed deer into transgenic mice expressing the hamster prion protein gene, cwd was subsequently passaged into syrian golden hamsters. in one passa ... | 2011 | 22174765 |
| soil-mediated prion transmission: is local soil-type a key determinant of prion disease incidence? | prion diseases, including chronic wasting disease (cwd) and scrapie, can be transmitted via indirect environmental routes. animals habitually ingest soil, and results from laboratory experiments demonstrate prions can bind to a wide range of soils and soil minerals, retain the ability to replicate, and remain infectious, indicating soil could serve as a reservoir for natural prion transmission and a potential prion exposure route for humans. preliminary epidemiological modeling suggests soil tex ... | 2012 | 22265680 |
| rapid detection of cwd prp: comparison of tests designed for the detection of bse or scrapie. | chronic wasting disease (cwd) is a transmissible spongiform encephalopathy (tse) mainly affecting cervids in north america. the accumulation of an abnormal form of host-encoded prion protein (prp(cwd) ) in the cns and lymphoid tissues is characteristic of the disease and known to be caused by pathogenic prion proteins (prp(res) ), which are thought to be transmitted mainly by contact with body fluids, such like saliva. species known to be naturally infected by cwd include rocky mountain elk (cer ... | 2011 | 22212828 |
| functional genomics approach for identification of molecular processes underlying neurodegenerative disorders in prion diseases. | prion diseases or transmissible spongiform encephalopathies (tses) are infectious neurodegenerative disorders leading to death. these include cresutzfeldt-jakob disease (cjd), familial, sporadic and variant cjd and kuru in humans; and animal tses include scrapie in sheep, bovine spongiform encephalopathy (bse) in cattle, chronic wasting disease (cwd) of mule deer and elk, and transmissible mink encephalopathy. all these tses share common pathological features such as accumulation of mis-folded p ... | 2012 | 23372423 |
| development of monoclonal antibodies against the abnormal prion protein isoform (prp(res)) associated with chronic wasting disease (cwd). | monoclonal antibodies (mabs) specific for the abnormal prion protein isoform (prp(res)) are indispensable for diagnosing chronic wasting disease (cwd). in this study, eight mabs were developed by immunizing prp knockout mice with recombinant elk prp and an immunogenic prp peptide. the reactivity of the mabs to recombinant prp and the prp peptide was measured, and their isotypes were subsequently determined. among them, four mabs (b85-05, b85-08, b85-12, and b77-75) were shown by western blotting ... | 2012 | 23271186 |
| association mapping of genetic risk factors for chronic wasting disease in wild deer. | chronic wasting disease (cwd) is a fatal transmissible spongiform encephalopathy affecting north american cervids. we assessed the feasibility of association mapping cwd genetic risk factors in wild white-tailed deer (odocoileus virginianus) and mule deer (odocoileus hemionus) using a panel of bovine microsatellite markers from three homologous deer linkage groups predicted to contain candidate genes. these markers had a low cross-species amplification rate (27.9%) and showed weak linkage disequ ... | 2012 | 23467626 |
| current perspectives in transfusion-transmitted infectious diseases: emerging and re-emerging infections. | in august 2009, a group from the aabb (stramer et al., transfusion 2009;99:1s-29s, emerging infectious disease agents and their potential threat to transfusion safety; http://www.aabb.org/resources/bct/eid/pages/default.aspx) published a supplement to transfusion that reviewed emerging infectious disease (eid) agents that pose a real or theoretical threat to transfusion safety, but for which an existing effective intervention is lacking. the necessary attributes for transfusion transmission were ... | 2014 | 25210533 |
| could avian scavengers translocate infectious prions to disease-free areas initiating new foci of chronic wasting disease? | mechanisms for the spread of transmissible spongiform encephalopathy diseases, including chronic wasting disease (cwd) in north american cervids, are incompletely understood, but primary routes include horizontal and environmental transmission. birds have been identified as potential vectors for a number of diseases, where they ingest or are exposed to infected material and later shed the disease agent in new areas after flying substantial distances. we recently identified american crows (corvus ... | 2013 | 23822910 |
| prion diseases as transmissible zoonotic diseases. | prion diseases, also called transmissible spongiform encephalopathies (tses), lead to neurological dysfunction in animals and are fatal. infectious prion proteins are causative agents of many mammalian tses, including scrapie (in sheep), chronic wasting disease (in deer and elk), bovine spongiform encephalopathy (bse; in cattle), and creutzfeldt-jakob disease (cjd; in humans). bse, better known as mad cow disease, is among the many recently discovered zoonotic diseases. bse cases were first repo ... | 0 | 24159531 |
| gene expression alterations in rocky mountain elk infected with chronic wasting disease. | chronic wasting disease (cwd) is an invariably fatal neurologic disease that naturally infects mule deer, white tailed deer and elk. the understanding of cwd neurodegeneration at a molecular level is very limited. in this study, microarray analysis was performed to determine changes in the gene expression profiles in six different tissues including brain, midbrain, thalamus, spleen, rpln and tonsil of cwd-infected elk in comparison to non-infected healthy elk, using 24,000 bovine specific oligo ... | 2012 | 22561165 |
| relationships between prpsc stability and incubation time for united states scrapie isolates in a natural host system. | transmissible spongiform encephalopathies (tses), including scrapie in sheep (ovis aries), are fatal neurodegenerative diseases caused by the misfolding of the cellular prion protein (prp(c)) into a â-rich conformer (prp(sc)) that accumulates into higher-order structures in the brain and other tissues. distinct strains of tses exist, characterized by different pathologic profiles upon passage into rodents and representing distinct conformations of prp(sc). one biochemical method of distinguishin ... | 2012 | 22916207 |
| inactivation of prions and amyloid seeds with hypochlorous acid. | hypochlorous acid (hocl) is produced naturally by neutrophils and other cells to kill conventional microbes in vivo. synthetic preparations containing hocl can also be effective as microbial disinfectants. here we have tested whether hocl can also inactivate prions and other self-propagating protein amyloid seeds. prions are deadly pathogens that are notoriously difficult to inactivate, and standard microbial disinfection protocols are often inadequate. recommended treatments for prion decontami ... | 2016 | 27685252 |
| mule deer spatial association patterns and potential implications for transmission of an epizootic disease. | animal social behaviour can have important effects on the long-term dynamics of diseases. in particular, preferential spatial relationships between individuals can lead to differences in the rates of disease spread within a population. we examined the concurrent influence of genetic relatedness, sex, age, home range overlap, time of year, and prion disease status on proximal associations of adult rocky mountain mule deer (odocoileus hemionus hemionus) in a chronic wasting disease endemic area. w ... | 2017 | 28388681 |
| when mechanism matters: bayesian forecasting using models of ecological diffusion. | ecological diffusion is a theory that can be used to understand and forecast spatio-temporal processes such as dispersal, invasion, and the spread of disease. hierarchical bayesian modelling provides a framework to make statistical inference and probabilistic forecasts, using mechanistic ecological models. to illustrate, we show how hierarchical bayesian models of ecological diffusion can be implemented for large data sets that are distributed densely across space and time. the hierarchical baye ... | 2017 | 28371055 |
| high prevalence of prion protein genotype associated with resistance to chronic wasting disease in one alberta woodland caribou population. | chronic wasting disease (cwd) is a prion disease found in deer, elk and moose in north america and since recently, wild reindeer in norway. caribou are at-risk to encounter cwd in areas such as alberta, canada, where the disease spreads toward caribou habitats. cwd susceptibility is modulated by species-specific polymorphisms in the prion protein gene (prnp). we sequenced prnp of woodland caribou from 9 albertan populations. in one population (chinchaga) a significantly higher frequency of the 1 ... | 2017 | 28350512 |
| are brazilian cervids at risk of prion diseases? | prion diseases are neurodegenerative fatal disorders that affect human and non-human mammals. chronic wasting disease (cwd) is a prion disease of cervids regarded as a public health problem in north america, and polymorphisms at specific codons in the prnp gene are associated with this disease. to assess the potential cwd susceptibility of south american free-ranging deer, the presence of these polymorphisms was examined in mazama gouazoubira, ozotoceros bezoarticus and blastocerus dichotomus. d ... | 2017 | 28281927 |