| elucidation of endemic neurodegenerative diseases--a commentary. | recent investigations of scrapie, creutzfeldt-jakob disease (cjd), and chronic wasting disease (cwd) clusters in iceland, slovakia and colorado, respectively, have indicated that the soil in these regions is low in copper and higher in manganese, and it has been well-known that patients of als or parkinson's disease were collectively found in the new guinea and papua islands, where the subterranean water (drinking water) contains much al3+ and mn2+ ions. above facts suggest that these neurodegen ... | 2003 | 14577644 |
| prion gene sequence variation within diverse groups of u.s. sheep, beef cattle, and deer. | prions are proteins that play a central role in transmissible spongiform encephalopathies in a variety of mammals. among the most notable prion disorders in ungulates are scrapie in sheep, bovine spongiform encephalopathy in cattle, and chronic wasting disease in deer. single nucleotide polymorphisms in the sheep prion gene ( prnp) have been correlated with susceptibility to natural scrapie in some populations. similar correlations have not been reported in cattle or deer; however, characterizat ... | 2003 | 14722726 |
| a processed pseudogene contributes to apparent mule deer prion gene heterogeneity. | pathogenesis and transmission of the prion disorders (transmissible spongiform encephalopathies, tses) are mediated by a modified isoform of the prion protein (prp). prion protein gene (prnp) alleles associated with relative susceptibility to tse have been identified in sheep, humans and possibly elk. comparable data have not been derived for mule deer, a species susceptible to the tse chronic wasting disease (cwd). initial analysis of the open reading frame (orf) in exon 3 of the mule deer prnp ... | 2004 | 14729275 |
| bovine spongiform encephalopathy in the united states--an epidemiologist's view. | | 2004 | 14762179 |
| transmission of transmissible mink encephalopathy to raccoons (procyon lotor) by intracerebral inoculation. | to determine the transmissibility of transmissible mink encephalopathy (tme) agent to raccoons and to provide information about clinical course, lesions, and suitability of currently used diagnostic procedures for detection of transmissible spongiform encephalopathies (tses) in raccoons, 4 raccoon kits were inoculated intracerebrally with a brain suspension from mink experimentally infected with tme. one uninoculated raccoon kit served as a control. all 4 animals in the tme-inoculated group show ... | 2004 | 14974848 |
| chronic wasting disease--prion disease in the wild. | | 2004 | 15094817 |
| polymorphisms in the prion precursor functional gene but not the pseudogene are associated with susceptibility to chronic wasting disease in white-tailed deer. | chronic wasting disease (cwd) status and prp genotypes were determined for a group of 133 wild white-tailed deer in a 780 acre enclosure in western nebraska, usa. approximately half of the deer tested showed evidence of prpd in the brainstem or lymphoid tissues. four prnp alleles encoding amino acid substitutions were identified, with substitutions at residues 95 (q-->h), 96 (g-->s) or 116 (a-->g), each with serine (s) at residue 138. in addition, a processed pseudogene with two alleles encoding ... | 2004 | 15105552 |
| preclinical deposition of pathological prion protein prpsc in muscles of hamsters orally exposed to scrapie. | recently, pathological prion protein prp(sc), the putative key constituent of infectious agents causing transmissible spongiform encephalopathies (tses), was found in muscles of rodents experimentally infected with scrapie and in patients with creutzfeldt-jakob disease (cjd). for the assessment of risk scenarios originating from these findings (e.g., alimentary transmission of pathogens associated with bovine spongiform encephalopathy [bse] and chronic wasting disease [cwd] via tainted beef and ... | 2004 | 15146244 |
| prion protein and the molecular features of transmissible spongiform encephalopathy agents. | transmissible spongiform encephalopathy (tse) diseases, or prion diseases, are neurodegenerative diseases found in a number of mammals, including man. although they are generally rare, tses are always fatal, and as of yet there are no practical therapeutic avenues to slow the course of disease. the epidemic of bovine spongiform encephalopathy (bse) in the uk greatly increased the awareness of tse diseases. although it appears that bse has not spread to north america, chronic wasting disease (cwd ... | 2004 | 15148986 |
| chronic wasting disease of cervids. | chronic wasting disease (cwd) has recently emerged in north america as an important prion disease of captive and free-ranging cervids (species in the deer family). cwd is the only recognized transmissible spongiform encephalopathy (tse) affecting free-ranging species. three cervid species, mule deer (odocoileus hemionus), white-tailed deer (o. virginianus), and rocky mountain elk (cervus elaphus nelsoni), are the only known natural hosts of cwd. endemic cwd is well established in southern wyomin ... | 2004 | 15148993 |
| president walther encourages federal funding to battle cwd. | | 2004 | 15154714 |
| molecular aspects of disease pathogenesis in the transmissible spongiform encephalopathies. | the transmissible spongiform encephalopathy (tse) diseases are a group of rare, fatal, and transmissible neurodegenerative diseases that include kuru and creutzfeldt-jakob disease (cjd) in humans, scrapie in sheep, transmissible mink encephalopathy (tme), and chronic wasting disease (cwd) in mule deer and elk. over the last 20 yr, they have gone from a fascinating but relatively obscure group of diseases to one that is a major agricultural and economic problem as well as a threat to human health ... | 2004 | 15156065 |
| prion disease and alzheimer's disease: pathogenic overlap. | prion diseases are widely recognized for their transmissibility, and it is this feature that has been studied most extensively. in recent years, public health concerns over the transmission of animal forms of prion disease, such as bovine spongiform encephalopathy and chronic wasting disease, to humans has only augmented the notion that prion diseases are primarily infectious. yet within the spectrum of human prion diseases, often overlooked is the fact that the overwhelming majority of cases ar ... | 2004 | 15190676 |
| transmissible spongiform encephalopathies in ruminants. | transmissible spongiform encephalopathies (tses) have been observed in north american sheep, cervidae, and cattle. the causative agent of tses seems to be proteins that induce a conformational change in normal host proteins. common clinical signs oftses include chronic weight loss and deteriorating neurologic function. mechanisms of tse transmission vary among host species. the diagnostic approach for tses of ruminants is presented,and the history and justification of regulatory programs to cont ... | 2004 | 15203228 |
| chronic wasting disease in canada: part 1. | the purpose of part 1 is to provide an overview of published literature (1980-2002) on chronic wasting disease (cwd) to inform canadian readers about the disease and to explain canadian regulatory approaches to the surveillance and control of cwd. much of the scientific information is drawn from american publications obtained from internet searches in pubmed and medline databases. the following keywords were used: chronic wasting disease, prion, diagnosis, transmissible spongiform encephalopathi ... | 2004 | 15206588 |
| chronic wasting disease and potential transmission to humans. | chronic wasting disease (cwd) of deer and elk is endemic in a tri-corner area of colorado, wyoming, and nebraska, and new foci of cwd have been detected in other parts of the united states. although detection in some areas may be related to increased surveillance, introduction of cwd due to translocation or natural migration of animals may account for some new foci of infection. increasing spread of cwd has raised concerns about the potential for increasing human exposure to the cwd agent. the f ... | 2004 | 15207045 |
| environmental sources of prion transmission in mule deer. | whether transmission of the chronic wasting disease (cwd) prion among cervids requires direct interaction with infected animals has been unclear. we report that cwd can be transmitted to susceptible animals indirectly, from environments contaminated by excreta or decomposed carcasses. under experimental conditions, mule deer (odocoileus hemionus) became infected in two of three paddocks containing naturally infected deer, in two of three paddocks where infected deer carcasses had decomposed in s ... | 2004 | 15207049 |
| linking chronic wasting disease to scrapie by comparison of spiroplasma mirum ribosomal dna sequences. | transmissible spongiform encephalopathies (tse) are fatal neurodegenerative diseases of man and animals and are transmitted by a filterable pathogen whose identity is currently unresolved. our data indicates that spiroplasma, a wall-less bacterium, is involved in the pathogenesis of tse. we searched for spiroplasma ribosomal gene sequences in 10 scrapie-infected sheep brains and 10 normal sheep brains, 7 cervid samples infected with chronic wasting disease (cwd), and 7 normal cervid brains. dna ... | 2004 | 15215050 |
| unicorn tapestries, horned animals, and prion disease. | | 2004 | 15224680 |
| elevated silver, barium and strontium in antlers, vegetation and soils sourced from cwd cluster areas: do ag/ba/sr piezoelectric crystals represent the transmissible pathogenic agent in tses? | high levels of silver (ag), barium (ba) and strontium (sr) and low levels of copper (cu) have been measured in the antlers, soils and pastures of the deer that are thriving in the chronic wasting disease (cwd) cluster zones in north america in relation to the areas where cwd and other transmissible spongiform encephalopathies (tses) have not been reported. the elevations of ag, ba and sr were thought to originate from both natural geochemical and artificial pollutant sources--stemming from the c ... | 2004 | 15236778 |
| progress and problems in the biology, diagnostics, and therapeutics of prion diseases. | the term "prion" was introduced by stanley prusiner in 1982 to describe the atypical infectious agent that causes transmissible spongiform encephalopathies, a group of infectious neurodegenerative diseases that include scrapie in sheep, creutzfeldt-jakob disease in humans, chronic wasting disease in cervids, and bovine spongiform encephalopathy in cattle. over the past twenty years, the word "prion" has been taken to signify various subtly different concepts. in this article, we refer to the pri ... | 2004 | 15254579 |
| transmission of sheep scrapie to elk (cervus elaphus nelsoni) by intracerebral inoculation: final outcome of the experiment. | this is a final report of an experimental transmission of sheep scrapie agent by intracerebral inoculation to rocky mountain elk (cervus elaphus nelsoni). it documents results obtained in experimental (n = 6) and control (n = 2) elk. during the first 2 years postinoculation (pi), 3 animals died or were euthanized because of infection or injuries other than spongiform encephalopathy (se). in years 3 and 4 pi, 3 other inoculated elk died after brief terminal neurological episodes. necropsy of thes ... | 2004 | 15305743 |
| epidemiology of chronic wasting disease in captive white-tailed and mule deer. | the natural occurrence of chronic wasting disease (cwd) in a 1993 cohort of captive white-tailed deer (odocoileus virginianus) afforded the opportunity to describe epidemic dynamics in this species and to compare dynamics with those seen in contemporary cohorts of captive mule deer (o. hemionus) also infected with cwd. the overall incidence of clinical cwd in white-tailed deer was 82% (nine of 11) among individuals that survived >15 mo. affected white-tailed deer died or were killed because of t ... | 2004 | 15362835 |
| variable patterns of distribution of prp(cwd) in the obex and cranial lymphoid tissues of rocky mountain elk (cervus elaphus nelsoni) with subclinical chronic wasting disease. | sections of medulla oblongata, taken at the level of the obex, palatine tonsil and medial retropharyngeal lymph node from 10,269 captive rocky mountain elk (cervus elaphus nelsoni), were examined by immunohistochemical staining with monoclonal antibody for the prion protein associated with the transmissible spongiform encephalopathy of cervids, chronic wasting disease (prp(cwd)). the protein was detected in 226 of them. on the basis of the anatomical location of the deposits in the brainstem of ... | 2004 | 15478500 |
| the first canadian indigenous case of bovine spongiform encephalopathy (bse) has molecular characteristics for prion protein that are similar to those of bse in the united kingdom but differ from those of chronic wasting disease in captive elk and deer. | brain tissue from a case of bovine spongiform encephalopathy (bse) from alberta was subjected to a western immunoblotting technique to ascertain the molecular profile of any disease-specific, abnormal prion protein, that is, prion protein that is protease-resistant (prp(res)). this technique can discriminate between isolates from bse, ovine scrapie, and sheep experimentally infected with bse. isolates of brain tissue from the bse case in alberta, 3 farmed elk with chronic wasting disease (cwd) f ... | 2004 | 15532881 |
| transmission of prions from mule deer and elk with chronic wasting disease to transgenic mice expressing cervid prp. | we generated mice expressing cervid prion protein to produce a transgenic system simulating chronic wasting disease (cwd) in deer and elk. while normal mice were resistant to cwd, these transgenic mice uniformly developed signs of neurological dysfunction approximately 230 days following intracerebral inoculation with four cwd isolates. inoculated transgenic mice homozygous for the transgene array developed disease after approximately 160 days. the brains of sick transgenic mice exhibited widesp ... | 2004 | 15542685 |
| infections of the nervous system. | | 2005 | 15620853 |
| prion protein nmr structures of elk and of mouse/elk hybrids. | the nmr structure of the recombinant elk prion protein (eprp), which represents the cellular isoform (eprpc) in the healthy organism, is described here. as anticipated from the highly conserved amino acid sequence, eprpc has the same global fold as other mammalian prion proteins (prps), with a flexibly disordered "tail" of residues 23-124 and a globular domain 125-226 with three alpha-helices and a short antiparallel beta-sheet. however, eprpc shows a striking local structure variation when comp ... | 2005 | 15647363 |
| the public health impact of prion diseases. | several prion disease-related human health risks from an exogenous source can be identified in the united states, including the iatrogenic transmission of creutzfeldt-jakob disease (cjd), the possible occurrence of variant cjd (vcjd), and potential zoonotic transmission of chronic wasting disease (cwd). although cross-species transmission of prion diseases seems to be limited by an apparent "species barrier," the occurrence of bovine spongiform encephalopathy (bse) and its transmission to humans ... | 2005 | 15760286 |
| surveillance of chronic wasting disease in sika deer, cervus nippon, from tokachi district in hokkaido. | surveillance of chronic wasting disease (cwd) was conducted by performing western blot analysis of tissue samples from 136 sika deer (cervus nippon) killed by hunters in the tokachi district of hokkaido island. no prion protein (prpsc) associated with cwd was detected in any of the samples. to assess amino acid polymorphisms of the sika deer prp gene, nucleotide sequencing of the prp gene was performed. the only amino acid polymorphisms detected were 3 silent mutations at nucleotide positions 63 ... | 2005 | 15805745 |
| prion disease: a deadly disease for protein misfolding. | an infectious particle, termed prion, composed largely and perhaps solely of a single protein, is the likely causative agent of prion disease. it produces lethal decline of cognitive and motor function. the responsible protein arrives at a pathogenic state by misfolding from a normal form that has ubiquitous tissue distribution. prion diseases are often called spongiform encephalopathies. probably most mammalian species develop these diseases. specific examples in various animals are -scrapie, t ... | 2005 | 15853695 |
| prion biology relevant to bovine spongiform encephalopathy. | bovine spongiform encephalopathy (bse) and chronic wasting disease (cwd) of deer and elk are a threat to agriculture and natural resources, as well as a human health concern. both diseases are transmissible spongiform encephalopathies (tse), or prion diseases, caused by autocatalytic conversion of endogenously encoded prion protein (prp) to an abnormal, neurotoxic conformation designated prpsc. most mammalian species are susceptible to tse, which, despite a range of species-linked names, is caus ... | 2005 | 15890824 |
| risk analysis of ectoparasites acting as vectors for chronic wasting disease. | prion diseases are rare neurodegenerative diseases of humans and animals with a lethal evolution. animal prion infections, such as chronic wasting disease (cwd) and scrapie (sheep) have shown a pattern of horizontal transmission. cwd is an endemic disease that has been affecting thousands of domestic and wild cervids in us for the last three decades. the mode of contamination is not known, although direct contact between infected and non-infected animals via saliva, urine and feces have been con ... | 2005 | 15893117 |
| cwd found in new york. | | 2005 | 15906554 |
| experimental transmission of chronic wasting disease agent from mule deer to cattle by the intracerebral route. | this communication reports final observations on experimental transmission of chronic wasting disease (cwd) from mule deer to cattle by the intracerebral route. thirteen calves were inoculated intracerebrally with brain suspension from mule deer naturally affected with cwd. three other calves were kept as uninoculated controls. the experiment was terminated 6 years after inoculation. during that time, abnormal prion protein (prp(res)) was demonstrated in the central nervous system (cns) of 5 cat ... | 2005 | 15945388 |
| usda takes steps to battle cwd. | | 2005 | 15989170 |
| bovine spongiform encephalopathy, multiple sclerosis, and creutzfeldt-jakob disease are probably autoimmune diseases evoked by acinetobacter bacteria. | bovine spongiform encephalopathy (bse) belongs to a group of conditions named together as transmissible spongiform encephalopathies (tse). they are fatal neurodegenerative diseases that include "scrapie" in sheep, creutzfeldt-jakob disease (cjd) and kuru in humans, and chronic wasting disease in deers. bse-affected animals suffer from "hindquarters" paralysis, which is also one of the main features of "experimental allergic encephalomyelitis" (eae). eae is considered an animal model of multiple ... | 2005 | 16014559 |
| low frequency of prp genotype 225sf among free-ranging mule deer (odocoileus hemionus) with chronic wasting disease. | the prion protein (prp) gene was characterized in 1482 free-ranging mule deer (odocoileus hemionus) from wyoming and colorado. using dna sequences from 363 deer, dimorphisms at codons 20 (aspartate/glycine) and 225 [serine (s)/phenylalanine (f)] were found; silent changes occurred at codons 131 (tyrosine) and 247 (isoleucine). the remaining samples were surveyed for codon 225 genotype and all were characterized for chronic wasting disease (cwd) infection status. a total of 112 deer with the geno ... | 2005 | 16033959 |
| epidemiology of chronic wasting disease in free-ranging mule deer: spatial, temporal, and demographic influences on observed prevalence patterns. | we analyzed chronic wasting disease (cwd) prevalence data from mule deer populations in northcentral colorado, usa, to discern the likely influences of temporal, spatial, and demographic factors on patterns observed in naturally infected populations. in addition to reaffirming spatial heterogeneity among wintering mule deer subpopulations, we report marked differences in cwd prevalence by sex and age groups as well as clear local trends of increasing prevalence over a 7-yr period. prevalence of ... | 2005 | 16107661 |
| bibliography. current world literature. nutrition in wasting disease. | | 2005 | 16108098 |
| [update on transmissible spongiform subacute encephalopathies (tsse)]. | this update concerns human and ruminant transmissible spongiform subacute encephalopathies (tsse). the latest data on variant creutzfeldt-jakob disease confirm that new cases are less frequent than feared some years ago, but subclinical carriers could be a source of iatrogenic infection. the macaque is a good model of human oral transmission of bovine spongiform encephalopathy (bse). the latest data on bse in europe confirm the effectiveness of precautionary measures taken in 1996 and 2000. conc ... | 2005 | 16114866 |
| chronic wasting disease of elk: transmissibility to humans examined by transgenic mouse models. | chronic wasting disease (cwd), a prion disease affecting free-ranging and captive cervids (deer and elk), is widespread in the united states and parts of canada. the large cervid population, the popularity of venison consumption, and the apparent spread of the cwd epidemic are likely resulting in increased human exposure to cwd in the united states. whether cwd is transmissible to humans, as has been shown for bovine spongiform encephalopathy (the prion disease of cattle), is unknown. we generat ... | 2005 | 16135751 |
| additional cases of chronic wasting disease in imported deer in korea. | chronic wasting disease (cwd), which had previously occurred only in the u.s.a. and canada, broke out in a farm at chungbuk, korea from imported canadian deer (aug. 8, 2001). cwd distribution, through surveillance and epidemiologic investigations, was reported for 93 deer (43 from the cwd originating farm and 50 imported with the cwd originating farm's deer) out of 144 deer (72 from the cwd originating farm and 72 imported with the cwd originating farm's deer) that were breeding at 30 different ... | 2005 | 16141661 |
| chronic wasting disease. | chronic wasting disease (cwd) is a unique transmissible spongiform encephalopathy (tse) of mule deer (odocoileus hemionus), white-tailed deer (o. virginianus), and rocky mountain elk (cervus elaphus nelsoni). the natural history of cwd is incompletely understood, but it differs from scrapie and bovine spongiform encephalopathy (bse) by virtue of its occurrence in nondomestic and free-ranging species. cwd has many features in common with scrapie, including early widespread distribution of disease ... | 2005 | 16145200 |
| locoweed (oxytropis sericea)-induced lesions in mule deer (odocoileius hemionus). | locoweed poisoning has been reported in wildlife, but it is unknown whether mule deer (odocoileius hemionus) are susceptible. in areas that are heavily infested with locoweed, deer and elk (cervus elaphus nelsoni) have developed a spongiform encephalopathy, chronic wasting disease (cwd). although these are distinct diseases, no good comparisons are available. the purpose of this study was to induce and describe chronic locoweed poisoning in deer and compare it with the lesions of cwd. two groups ... | 2005 | 16145203 |
| prion diseases. | prion diseases are degenerative disorders of the nervous system caused by transmissible particles that contain a pathogenic isoform of the prion protein, a normal constituent of cell membranes. the most common human prion disease is creutzfeldt-jakob disease (cjd). most cases are sporadic with unknown mode of transmission, 10-15% of cases are inherited, and a small number have been transmitted by medical procedures. the spread of human prion diseases through consumption of infected material has ... | 2005 | 16168932 |
| evaluation of western blotting methods using samples with or without sodium phosphotungstic acid precipitation for diagnosis of scrapie and chronic wasting disease. | the purpose of this study was to enhance the sensitivity of the western blot (wb) test for use as an alternative and confirmatory method for the diagnosis of scrapie and chronic wasting disease (cwd) in canada by comparing 2 sample preparation procedures: an abnormal prion protein (prpsc) concentration procedure using sodium phosphotungstic acid (pta) precipitation and a procedure using crude sample without precipitation. a total of 100 cerebrum samples (52 sheep and 48 elk), including 66 negati ... | 2005 | 16187549 |
| spiroplasma as a candidate agent for the transmissible spongiform encephalopathies. | the recovery of a novel spiroplasma sp. from brain tissues from sheep with scrapie, cervids with chronic wasting disease, and from patients with creutzfeldt-jakob disease through passage through embryonated eggs has raised the issue of the role of spiroplasma in the transmissible spongiform encephalopathies (tse). in this review, we have inserted into an epidemiologic infection model evidence accumulated over the past 30 years showing involvement of spiroplasma infection in tse. these data suppo ... | 2005 | 16215454 |
| interspecies transmission of chronic wasting disease prions to squirrel monkeys (saimiri sciureus). | chronic wasting disease (cwd) is an emerging prion disease of deer and elk. the risk of cwd transmission to humans following exposure to cwd-infected tissues is unknown. to assess the susceptibility of nonhuman primates to cwd, two squirrel monkeys were inoculated with brain tissue from a cwd-infected mule deer. the cwd-inoculated squirrel monkeys developed a progressive neurodegenerative disease and were euthanized at 31 and 34 months postinfection. brain tissue from the cwd-infected squirrel m ... | 2005 | 16227298 |
| first results of chronic wasting disease (cwd) surveillance in the south-eastern part of belgium. | chronic wasting disease (cwd) has not been reported in europe, whereas it is considered to be enzootic in free-ranging mule deer, rocky mountain elk and white-tailed deer in the area of colorado, wyoming, and nebraska, and new foci of cwd have been detected in other parts of the united states. however, no large-scale active epidemiosurveillance of european wild cervids has been installed in europe. in accordance with the opinion of the european scientific steering committee, a preliminary (activ ... | 2005 | 16238109 |
| molecular evolution of the sheep prion protein gene. | transmissible spongiform encephalopathies (tses) are infectious, fatal neurodegenerative diseases characterized by aggregates of modified forms of the prion protein (prp) in the central nervous system. well known examples include variant creutzfeldt-jakob disease (vcjd) in humans, bse in cattle, chronic wasting disease in deer and scrapie in sheep and goats. in humans, sheep and deer, disease susceptibility is determined by host genotype at the prion protein gene (prnp). here i examine the molec ... | 2005 | 16243700 |
| relative vulnerability of chronic wasting disease infected mule deer to vehicle collisions. | we estimated chronic wasting disease (cwd) prevalence among vehicle-killed mule deer (odocoileus hemionus) in select data analysis units (daus) in northern colorado, usa, and compared these with estimated cwd prevalence among mule deer of the same sex sampled in the vicinity of collision sites to assess relative vulnerability of cwd-infected individuals to vehicle collisions. twenty-five of 171 vehicle-killed mule deer tested positive for cwd (overall prevalence=0.146, 95% confidence interval [c ... | 2005 | 16244060 |
| slow virus disease: deciphering conflicting data on the transmissible spongiform encephalopathies (tse) also called prion diseases. | the transmissible spongiform encephalopathies (tse) that manifest as creutzfeldt-jakob disease in humans, as scrapie in sheep and goats, mad cow disease in cattle, or chronic wasting disease in cervids (deer) represent a serious human health crisis and a significant economical problem. despite much research, the nature of the elusive pathogen directly involved with tse is currently unresolved. this article reviews current pathogen-cell plasma membrane properties, showing that the primary biochem ... | 2005 | 16276518 |
| second passage of sheep scrapie and transmissible mink encephalopathy (tme) agents in raccoons (procyon lotor). | to determine the transmissibility and pathogenicity of sheep scrapie and transmissible mink encephalopathy (tme) agents derived from raccoons (first passage), raccoon kits were inoculated intracerebrally with either tme (one source) or scrapie (two sources-each in separate groups of raccoons). two uninoculated raccoon kits served as controls. all animals in the tme-inoculated group developed clinical signs of neurologic dysfunction and were euthanatized between postinoculation month (pim) 6 and ... | 2005 | 16301585 |
| chronic wasting disease of elk and deer and creutzfeldt-jakob disease: comparative analysis of the scrapie prion protein. | chronic wasting disease (cwd), a transmissible prion disease that affects elk and deer, poses new challenges to animal and human health. although the transmission of cwd to humans has not been proven, it remains a possibility. if this were to occur, it is important to know whether the "acquired" human prion disease would show a phenotype including the scrapie prion protein (prp(sc)) features that differ from those associated with human sporadic prion disease. in this study, we have compared the ... | 2006 | 16338930 |
| species barriers in prion diseases--brief review. | transmissible spongiform encephalopathies (tses or prion diseases) are neurological disorders associated with the aggregation of a pathologic isoform of a host-encoded protein, termed prion protein (prp). the pathologic isoform of prp, termed prp(sc), is a major constituent of the infectious agent. tse diseases are characterized by neurodegenerative failure and inevitable morbidity. bovine spongiform encephalopathy (bse) has been transmitted from cattle to humans to cause a new variant of creutz ... | 2005 | 16355873 |
| spongiform diseases. after the crisis: more questions about prions. | | 2005 | 16357237 |
| inhibition of protease-resistant prion protein formation in a transformed deer cell line infected with chronic wasting disease. | chronic wasting disease (cwd) is an emerging transmissible spongiform encephalopathy (prion disease) of north american cervids, i.e., mule deer, white-tailed deer, and elk (wapiti). to facilitate in vitro studies of cwd, we have developed a transformed deer cell line that is persistently infected with cwd. primary cultures derived from uninfected mule deer brain tissue were transformed by transfection with a plasmid containing the simian virus 40 genome. a transformed cell line (mdb) was exposed ... | 2006 | 16378962 |
| a retrospective study of the causes of morbidity and mortality in farmed elk (cervus elaphus). | a survey of north american farmed elk (cervus elaphus) producers was performed to determine the causes of sickness and mortality in farmed elk and to estimate mortality rates. records over a 10-year period from 8 north american veterinary diagnostic pathology laboratories were also examined and summarized. the primary diagnosis for each record was used to classify diseases into categories such as parasitic, infectious, toxicological, and neoplastic. nonspecific trauma was the most frequently rep ... | 2005 | 16422063 |
| experimental second passage of chronic wasting disease (cwd(mule deer)) agent to cattle. | to compare clinicopathological findings in first and second passage chronic wasting disease (cwd(mule deer)) in cattle, six calves were inoculated intracerebrally with brain tissue derived from a first-passage cwd-affected calf in an earlier experiment. two uninoculated calves served as controls. the inoculated animals began to lose both appetite and weight 10-12 months later, and five subsequently developed clinical signs of central nervous system (cns) abnormality. by 16.5 months, all cattle h ... | 2006 | 16423572 |
| prions in skeletal muscles of deer with chronic wasting disease. | the emergence of chronic wasting disease (cwd) in deer and elk in an increasingly wide geographic area, as well as the interspecies transmission of bovine spongiform encephalopathy to humans in the form of variant creutzfeldt jakob disease, have raised concerns about the zoonotic potential of cwd. because meat consumption is the most likely means of exposure, it is important to determine whether skeletal muscle of diseased cervids contains prion infectivity. here bioassays in transgenic mice exp ... | 2006 | 16439622 |
| tonsillar biopsy test for chronic wasting disease: two sampling approaches in mule deer and white-tailed deer. | preclinical antemortem testing of deer (odocoileus spp.) for chronic wasting disease (cwd) can be important for determining prevalence rates and removing infected individuals from wild populations. because samples with high numbers of tonsillar follicles are likely to provide earlier detection of cwd than samples with fewer follicles, the method of obtaining follicular samples may be critical when investigating disease prevalence. between january 2003 and january 2005, white-tailed deer (o. virg ... | 2005 | 16456177 |
| [muscular power of masticating muscles and mandibular osteoporosis]. | whereas the most powerful stimuli for bone formation is supposed to be a stretching of muscles, frost hm classified the effect of muscle on bone mineral density (bmd) into various types: 1. age-related loss of bone mineral density (bmd) is partly due to loss of muscular wasting, 2. the increase of bmd in obesity is due to the increase in muscular power to support the increased body weight and 3. the decrease of bmd in chronic wasting disease is partly due to the decrease in muscular power. likew ... | 2006 | 16465028 |
| diagnosis of transmissible spongiform encephalopathies in animals: a review. | transmissible spongiform encephalopathies (tses) in animals include, among others, bovine spongiform encephalopathy (bse), scrapie, chronic wasting disease, and atypical forms of prion diseases. diagnosis of tses is based on identification of characteristic lesions or on detection of the abnormal prion proteins in tissues, often by use of their partial proteinase k resistance property. correctly sampling of target tissues is of utmost importance as this has a considerable effect on test sensitiv ... | 2005 | 16475509 |
| first and second cattle passage of transmissible mink encephalopathy by intracerebral inoculation. | to compare clinicopathologic findings of transmissible mink encephalopathy (tme) with other transmissible spongiform encephalopathies (tse, prion diseases) that have been shown to be experimentally transmissible to cattle (sheep scrapie and chronic wasting disease [cwd]), two groups of calves (n = 4 each) were intracerebrally inoculated with tme agents from two different sources (mink with tme and a steer with tme). two uninoculated calves served as controls. within 15.3 months postinoculation, ... | 2006 | 16537929 |
| preliminary observations of genetic susceptibility of elk (cervus elaphus nelsoni) to chronic wasting disease by experimental oral inoculation. | to compare the genetic susceptibility of elk (cervus elaphus nelsoni) with various alleles of the prnp gene, which encodes the normal cellular prion protein, to chronic wasting disease (cwd), eight 8-month-old elk calves of 3 genotypes (2 132mm, 2 132lm, and 4 132ll) were orally dosed with cwd-infected brain material from elk. during postinoculation (pi) month 23, both 132mm elk had lost appetite, developed clinical signs of weight loss and central nervous system (cns) dysfunction, and were euth ... | 2006 | 16566268 |
| the expanding universe of prion diseases. | prions cause fatal and transmissible neurodegenerative disease. these etiological infectious agents are formed in greater part from a misfolded cell-surface protein called prp(c). several mammalian species are affected by the diseases, and in the case of "mad cow disease" (bse) the agent has a tropism for humans, with negative consequences for agribusiness and public health. unfortunately, the known universe of prion diseases is expanding. at least four novel prion diseases--including human dise ... | 2006 | 16609731 |
| comparison of two automated immunohistochemical procedures for the diagnosis of scrapie in domestic sheep and chronic wasting disease in north american white-tailed deer (odocoileus virginianus) and mule deer (odocoileus hemionus). | two commercially available automated immunohistochemistry platforms, ventana nexes and dakocytomation autostainer universal staining system, were compared for diagnosing sheep scrapie and cervid chronic wasting disease. both automated platforms used the same antiprion protein monoclonal primary antibodies, but different platform-specific linker and amplification reagents and procedures. duplicate sections of brainstem (at the level of the obex) and lymphoid tissue (retropharyngeal lymph node or ... | 2006 | 16617694 |
| abnormal prion protein in ectopic lymphoid tissue in a kidney of an asymptomatic white-tailed deer experimentally inoculated with the agent of chronic wasting disease. | chronic wasting disease (cwd), a transmissible spongiform encephalopathy (tse) of deer and elk, is one of a group of fatal, neurologic diseases that affect several mammalian species, including human beings. infection by the causative agent induces accumulations of an abnormal form of prion protein (prpres) in nervous and lymphoid tissues. this report documents the presence of prpres within ectopic lymphoid follicles in a kidney of a white-tailed deer that had been experimentally inoculated by th ... | 2006 | 16672586 |
| gene expression alterations in brains of mice infected with three strains of scrapie. | transmissible spongiform encephalopathies (tses) or prion diseases are fatal neurodegenerative disorders which occur in humans and various animal species. examples include creutzfeldt-jakob disease (cjd) in humans, bovine spongiform encephalopathy (bse) in cattle, chronic wasting disease (cwd) in deer and elk, and scrapie in sheep, and experimental mice. to gain insights into tse pathogenesis, we made and used cdna microarrays to identify disease-associated alterations in gene expression. brain ... | 2006 | 16700923 |
| prion protein polymorphisms in white-tailed deer influence susceptibility to chronic wasting disease. | the primary sequence of the prion protein affects susceptibility to transmissible spongiform encephalopathies, or prion diseases, in mice, sheep and humans. the prnp gene sequence of free-ranging, wisconsin white-tailed deer was determined and the prnp genotypes of chronic wasting disease (cwd)-positive and cwd-negative deer were compared. six amino acid changes were identified, two of which were located in pseudogenes. two alleles, a q-->k polymorphism at codon 226 and a single octapeptide repe ... | 2006 | 16760415 |
| the dutch famine of 1944-1945: a pathophysiological model of long-term consequences of wasting disease. | the tragic circumstances of the dutch hunger winter of 1944-1945 created a unique opportunity to study the relation between exposure to prenatal famine and health in adult life. this review addresses the literature on the effects of maternal malnutrition during the different periods of gestation and childhood on health in adult life. | 2006 | 16778567 |
| current and future molecular diagnostics for prion diseases. | it is now widely held that the infectious agents underlying the transmissible spongiform encephalopathies are prions, which are primarily composed of a misfolded, protease-resistant isoform of the host prion protein. untreatable prion disorders include some human diseases, such as creutzfeldt-jakob disease, and diseases of economically important animals, such as bovine spongiform encephalopathy (cattle) and chronic wasting disease (deer and elk). detection and diagnosis of prion disease (and pre ... | 2006 | 16824033 |
| linking chronic wasting disease to mule deer movement scales: a hierarchical bayesian approach. | observed spatial patterns in natural systems may result from processes acting across multiple spatial and temporal scales. although spatially explicit data on processes that generate ecological patterns, such as the distribution of disease over a landscape, are frequently unavailable, information about the scales over which processes operate can be used to understand the link between pattern and process. our goal was to identify scales of mule deer (odocoileus hemionus) movement and mixing that ... | 2006 | 16827000 |
| first chronic wasting disease (cwd) surveillance of roe deer (capreolus capreolus) in the northern part of belgium. | cases of chronic wasting disease (cwd) in wild cervids have yet not been reported in europe, whereas the disease is considered enzootic in free-ranging mule deer, rocky mountain elk and white-tailed deer in the area of colorado, wyoming, and nebraska. new foci of cwd continue to be detected in other parts of the united states. however, no large-scale active epidemiosurveillance of european wild cervids is yet installed in europe. in accordance with the opinion of the european scientific steering ... | 2006 | 16841567 |
| polymorphisms of the prion protein gene (prnp ) in alaskan moose (alces alces gigas). | | 2006 | 16879366 |
| reconstructing prions: fibril assembly from simple yeast to complex mammals. | with the epizootics of bovine spongiform encephalopathy (bse) in north american cattle, bse infections in goats, new forms of human creutzfeldt-jakob disease (cjd) and the spread of chronic wasting disease in north american deer and elk, one wonders whether we are gaining control over the transmissible spongiform encephalopathies (tses). although many basic scientific questions in the prion field remain hotly debated and unresolved [1], including the function of the cellular prion protein (prp), ... | 2005 | 16908997 |
| transmission of elk and deer prions to transgenic mice. | chronic wasting disease (cwd) is a fatal prion disease in deer and elk. unique among the prion diseases, it is transmitted among captive and free-ranging animals. to facilitate studies of the biology of cwd prions, we generated five lines of transgenic (tg) mice expressing prion protein (prp) from rocky mountain elk (cervus elaphus nelsoni), denoted tg(elkprp), and two lines of tg mice expressing prp common to white-tailed deer (odocoileus virginianus) and mule deer (odocoileus hemionus), denote ... | 2006 | 16940522 |
| myiasis as a risk factor for prion diseases in humans. | prion diseases are transmissible spongiform encephalopathies of humans and animals. the oral route is clearly associated with some prion diseases, according to the dissemination of bovine spongiform encephalopathy (bse or mad cow disease) in cattle and kuru in humans. however, other prion diseases such as scrapie (in sheep) and chronic wasting disease (cwd) (in cervids) cannot be explained in this way and are probably more associated with a pattern of horizontal transmission in both domestic and ... | 2006 | 16987255 |
| the fitting of general force-of-infection models to wildlife disease prevalence data. | researchers and wildlife managers increasingly find themselves in situations where they must deal with infectious wildlife diseases such as chronic wasting disease, brucellosis, tuberculosis, and west nile virus. managers are often charged with designing and implementing control strategies, and researchers often seek to determine factors that influence and control the disease process. all of these activities require the ability to measure some indication of a disease's foothold in a population a ... | 2006 | 16995636 |
| strain fidelity of chronic wasting disease upon murine adaptation. | chronic wasting disease (cwd), a prion disease of deer and elk, is highly prevalent in some regions of north america. the establishment of mouse-adapted cwd prions has proven difficult due to the strong species barrier between mice and deer. here we report the efficient transmission of cwd to transgenic mice overexpressing murine prp. all mice developed disease 500 +/- 62 days after intracerebral cwd challenge. the incubation period decreased to 228 +/- 103 days on secondary passage and to 162 + ... | 2006 | 17020952 |
| infectious prions in the saliva and blood of deer with chronic wasting disease. | a critical concern in the transmission of prion diseases, including chronic wasting disease (cwd) of cervids, is the potential presence of prions in body fluids. to address this issue directly, we exposed cohorts of cwd-naïve deer to saliva, blood, or urine and feces from cwd-positive deer. we found infectious prions capable of transmitting cwd in saliva (by the oral route) and in blood (by transfusion). the results help to explain the facile transmission of cwd among cervids and prompt caution ... | 2006 | 17023660 |
| prion protein in cardiac muscle of elk (cervus elaphus nelsoni) and white-tailed deer (odocoileus virginianus) infected with chronic wasting disease. | to investigate the possible presence of disease-associated prion protein (prp(d)) in striated muscle of chronic wasting disease (cwd)-affected cervids, samples of diaphragm, tongue, heart and three appendicular skeletal muscles from mule deer (odocoileus hemionus), white-tailed deer (odocoileus virginianus), elk (cervus elaphus nelsoni) and moose (alces alces shirasi) were examined by elisa, western immunoblot and immunohistochemistry (ihc). prp(d) was detected in samples of heart muscle from se ... | 2006 | 17030881 |
| patterns of prpcwd accumulation during the course of chronic wasting disease infection in orally inoculated mule deer (odocoileus hemionus). | patterns of abnormal prion protein (prp) accumulation during the course of chronic wasting disease (cwd) infection were studied and the distribution and timing of disease-associated prp (prp(cwd)) deposition and lesions in 19 mule deer (odocoileus hemionus) 90-785 days after oral inoculation were described. prp(cwd) deposition occurred relatively rapidly and widely in lymphoid tissues, later in central and peripheral nervous tissues and sporadically in a variety of tissues and organs in terminal ... | 2006 | 17030882 |
| the transmissible spongiform encephalopathies: emerging and declining epidemics. | tses (transmissible spongiform encephalopathies) are neurodegenerative diseases of various mammalian species, the best known of which include bse (bovine spongiform encephalopathies) in cattle, cjd (creutzfeldt-jakob disease) in humans, scrapie in sheep and cwd (chronic wasting disease) in deer. this review examines the emergence of various tse strains and their transmission, and discusses disease surveillance and control. | 2006 | 17073774 |
| copper and the prion protein: methods, structures, function, and disease. | the transmissible spongiform encephalopathies (tses) arise from conversion of the membrane-bound prion protein from prp(c) to prp(sc). examples of the tses include mad cow disease, chronic wasting disease in deer and elk, scrapie in goats and sheep, and kuru and creutzfeldt-jakob disease in humans. although the precise function of prp(c) in healthy tissues is not known, recent research demonstrates that it binds cu(ii) in an unusual and highly conserved region of the protein termed the octarepea ... | 2007 | 17076634 |
| test for detection of disease-associated prion aggregate in the blood of infected but asymptomatic animals. | we have developed a sensitive in vitro assay for detecting disease-associated prion aggregates by combining an aggregation-specific enzyme-linked immunosorbent assay (as-elisa) with the fluorescent amplification catalyzed by t7 rna polymerase technique (factt). the new assay, named aggregation-specific factt (as-factt), is much more sensitive than as-elisa and could detect prion aggregates in the brain of mice as early as 7 days after an intraperitoneal inoculation of prp(sc). however, as-factt ... | 2007 | 17079434 |
| spatial epidemiology of chronic wasting disease in wisconsin white-tailed deer. | chronic wasting disease (cwd) is a fatal, emerging disease of cervids associated with transmissible protease-resistant prion proteins. the potential for cwd to cause dramatic declines in deer and elk populations and perceived human health risks associated with consuming cwd-contaminated venison have led wildlife agencies to embark on extensive cwd control programs, typically involving culling to reduce deer populations. we characterized the spatial distribution of cwd in white-tailed deer (odoco ... | 2006 | 17092889 |
| oral transmission of chronic wasting disease in captive shira's moose. | three captive shira's moose (alces alces shirasi) were orally inoculated with a single dose (5 g) of whole-brain homogenate prepared from chronic wasting disease (cwd)-affected mule deer (odocoileus hemionus). all moose died of causes thought to be other than cwd. histologic examination of one female moose dying 465 days postinoculation revealed spongiform change in the neuropil, typical of transmissible spongiform encephalopathy. immunohistochemistry staining for the proteinase-resistant isofor ... | 2006 | 17092895 |
| passage of chronic wasting disease prion into transgenic mice expressing rocky mountain elk (cervus elaphus nelsoni) prpc. | chronic wasting disease (cwd) of elk (cervus elaphus nelsoni) and mule deer (odocoileus hemionus) is one of three naturally occurring forms of prion disease, the others being creutzfeldt-jakob disease in humans and scrapie in sheep. in the last few decades, cwd has spread among captive and free-ranging cervids in 13 us states, two canadian provinces and recently in korea. the origin of the cwd agent(s) in cervids is not known. this study describes the development of a transgenic mouse line (tgel ... | 2006 | 17098997 |
| detection of prp(cwd) in postmortem rectal lymphoid tissues in rocky mountain elk (cervus elaphus nelsoni) infected with chronic wasting disease. | preclinical diagnostic tests for transmissible spongiform encephalopathies have been described for mule deer (odocoileus hemionus), using biopsy tissues of palatine tonsil, and for sheep, using lymphoid tissues from palatine tonsil, third eyelid, and rectal mucosa. the utility of examining the rectal mucosal lymphoid tissues to detect chronic wasting disease (cwd) was investigated in rocky mountain elk (cervus elaphus nelsoni), a species for which there is not a live-animal diagnostic test. post ... | 2006 | 17121082 |
| transmission of chronic wasting disease of mule deer to suffolk sheep following intracerebral inoculation. | to determine the transmissibility of chronic wasting disease (cwd) to sheep, 8 suffolk lambs of various prion protein genotypes (4 arq/arr, 3 arq/arq, 1 arq/vrq at codons 136, 154, and 171, respectively) were inoculated intracerebrally with brain suspension from mule deer with cwd (cwdmd). two other lambs were kept as noninoculated controls. within 36 months postinoculation (mpi), 2 inoculated animals became sick and were euthanized. only 1 sheep (euthanized at 35 mpi) showed clinical signs that ... | 2006 | 17121083 |
| brief review on the epidemiology of transmissible spongiform encephalopathies (tse). | transmissible spongiform encephalopathies (tse) form a group of human and animal diseases that share common features such as (a) distinct pathological lesions in the central nervous system, (b) transmissibility at least in experimental settings, and (c) a long incubation period. considerable differences exist in the host range of individual tses, their routes of transmission, and factors influencing the host susceptibility (such as genotype). the objective of this review was to briefly describe ... | 2007 | 17126962 |
| prions found in deer body fluids. | | 2006 | 17148717 |
| novel aspects of prions, their receptor molecules, and innovative approaches for tse therapy. | 1. prion diseases are a group of rare, fatal neurodegenerative diseases, also known as transmissible spongiform encephalopathies (tses), that affect both animals and humans and include bovine spongiform encephalopathy (bse) in cattle, scrapie in sheep, chronic wasting disease (cwd) in deer and elk, and creutzfeldt-jakob disease (cjd) in humans. tses are usually rapidly progressive and clinical symptoms comprise dementia and loss of movement coordination due to the accumulation of an abnormal iso ... | 2007 | 17151946 |
| the vagus nerve as a conduit for neuroinvasion, a diagnostic tool, and a therapeutic pathway for transmissible spongiform encephalopathies, including variant creutzfeld jacob disease. | it is hypothesised that the vagus nerve (cranial nerve x) is an important conduit for infective neuroinvasion during the incubation of certain transmissible spongiform encephalopathies (tses) including scrapie in sheep, variant creutzfeld jacob disease in humans, chronic wasting disease in deer, and bovine spongiform encephalopathy in cattle. presence of infection in the brainstem will disrupt normal function of this important region responsible for autonomic control of visceral function via the ... | 2007 | 17166667 |
| human prion disease and relative risk associated with chronic wasting disease. | the transmission of the prion disease bovine spongiform encephalopathy (bse) to humans raises concern about chronic wasting disease (cwd), a prion disease of deer and elk. in 7 colorado counties with high cwd prevalence, 75% of state hunting licenses are issued locally, which suggests that residents consume most regionally harvested game. we used colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease creutzfeldt-jakob disease (cjd). the rel ... | 2006 | 17176567 |
| dynamics of prion disease transmission in mule deer. | chronic wasting disease (cwd), a contagious prion disease of the deer family, has the potential to severely harm deer populations and disrupt ecosystems where deer occur in abundance. consequently, understanding the dynamics of this emerging infectious disease, and particularly the dynamics of its transmission, has emerged as an important challenge for contemporary ecologists and wildlife managers. although cwd is contagious among deer, the relative importance of pathways for its transmission re ... | 2006 | 17205898 |
| retrospective investigation of chronic wasting disease of cervids at the toronto zoo, 1973-2003. | the occurrence of chronic wasting disease (cwd) at the toronto zoo was investigated retrospectively, based on an examination of management, animal health, and postmortem records, and immunohistochemical studies. records of animal movements, clinical signs, and postmortem findings were examined for all cervids 1973-2003. all available samples of fixed, wax-embedded lymphoid or central nervous system tissue from cervids that died at the toronto zoo from 1973 to 2003, > 12 months of age, were teste ... | 2006 | 17217088 |
| chronic wasting disease. | until recently, chronic wasting disease of cervids, the only prion disease affecting wildlife, was believed to be geographically concentrated to colorado and wyoming within the united states. however, increased surveillance has unveiled several additional pockets of cwd-infected deer and elk in 12 additional states and 2 canadian provinces. deer and elk with cwd have extensive aggregates of prp(sc) not only in the central nervous system, but also in peripheral lymphoid tissues, skeletal muscle, ... | 2007 | 17223321 |