Publications
| Title | Abstract | Year Filter | PMID(sorted descending) Filter |
|---|
| impact of strong selection for the prp major gene on genetic variability of four french sheep breeds(open access publication). | effective selection on the prp gene has been implemented since october 2001 in all french sheep breeds. after four years, the arr "resistant" allele frequency increased by about 35% in young males. the aim of this study was to evaluate the impact of this strong selection on genetic variability. it is focussed on four french sheep breeds and based on the comparison of two groups of 94 animals within each breed: the first group of animals was born before the selection began, and the second, 3-4 ye ... | 2008 | 18990357 |
| a rapid accurate culture assay for infectivity in transmissible encephalopathies. | the molecular and structural features of infectious agents that cause cjd, scrapie and bse remain controversial. a major impediment for agent resolution is the very long and expensive animal assays of infectivity. it is crucial to develop a rapid and broadly applicable cell culture assay to titer and compare different tse agent strains. because we found gt1 hypothalamic cells, unlike neuroblastoma n2a clones, were highly susceptible to a variety of tse agents, and could stably produce high agent ... | 2008 | 18989813 |
| a mirna signature of prion induced neurodegeneration. | micrornas (mirnas) are small, non-coding rna molecules which are emerging as key regulators of numerous cellular processes. compelling evidence links mirnas to the control of neuronal development and differentiation, however, little is known about their role in neurodegeneration. we used microarrays and rt-pcr to profile mirna expression changes in the brains of mice infected with mouse-adapted scrapie. we determined 15 mirnas were de-regulated during the disease processes; mir-342-3p, mir-320, ... | 2008 | 18987751 |
| prp antibody binding-induced epitope modulation evokes immunocooperativity. | we have characterized the antibody-antigen binding events of the prion protein (prp) utilizing three new prp-specific monoclonal antibodies (mabs). the degree of immunoreactivity was dependent on the denaturation treatment with the combination of heat and sds resulting in the highest levels of epitope accessibility and antibody binding. interestingly however, this harsh denaturation treatment was not sufficient to completely and irreversibly abolish protein conformation. the mabs differed in the ... | 2008 | 18977037 |
| increased neurogenesis in brains of scrapie-infected mice. | persistent neurogenesis occurs in the adult brain throughout the life of all mammals. recent studies have shown that neurogenesis was increased in adult gerbil and rat brains after ischemia. neurogenesis has not been examined during neurodegenerative diseases such as scrapie. to investigate the regeneration of neurons after scrapie-infection, we infused 5-bromo-2'-deoxyuridine (brdu), a dna replication indicator, into both control and scrapie-infected mice. mice were sacrificed at 150 days post- ... | 2009 | 18973796 |
| ablation of prion protein immunoreactivity by heating in saturated calcium hydroxide. | prions, the infectious agents that cause transmissible spongiform encephalopathies (tses), are relatively resistant to destruction by physical, enzymatic, and chemical treatments. hydrolysis in boiling saturated calcium hydroxide (limewater) utilizes inexpensive chemicals to digest protein components of offal. the purpose of this work was to determine if incubating brain material from scrapie-infected sheep in near-boiling saturated calcium hydroxide solution (ca(oh)2) would abolish immunoreacti ... | 2008 | 18957103 |
| the early history of the transmissible spongiform encephalopathies exemplified by scrapie. | transmissible spongiform encephalopathies (tse) is a group of diseases that is unique in comprising disorders that can occur sporadically, are hereditary and/or infectious. the transmissible pathogen--the prion--is distinct from all other pathogens in being devoid of nucleic acids. during the elucidation of these disorders, many different--and contradictory--theories have been put forward. early researchers, mostly driven by the economic impact of these diseases on sheep farming, engaged in heav ... | 2008 | 18951958 |
| reduction of prion infectivity in packed red blood cells. | the link between a new variant form of creutzfeldt-jakob disease (vcjd) and the consumption of prion contaminated cattle meat as well as recent findings showing that vcjd can be transmitted by blood transfusion have raised public health concerns. currently, a reliable test to identify prions in blood samples is not available. the purpose of this study was to evaluate the possibility to remove scrapie prion protein (prp(sc)) and infectivity from red blood cell (rbc) suspensions by a simple washin ... | 2008 | 18851948 |
| frequency of prnp genotypes in common new zealand sheep breeds. | 2008 | 18849578 | |
| kuru likened to scrapie: the story remembered. | 2008 | 18849258 | |
| [newly discovered forms of prion diseases in ruminants]. | transmissible spongiform encephalopathies (tses), are fatal neurodegenerative diseases caused by unconventional agents, the prions. they are characterised by the accumulation in infected tissues of an abnormally folded form of the host-encoded prion protein (prp). this pathological form is partially resistant to protease digestion, leading to the production of so-called prp(res) fragments. different isolates from the same host species may show different eletrophoretic profiles, reflecting the ex ... | 2009 | 18848406 |
| accelerated prion replication in, but prolonged survival times of, prion-infected cxcr3-/- mice. | prion diseases have a significant inflammatory component. glia activation, which is associated with increased production of cytokines and chemokines, may play an important role in disease development. among the chemokines upregulated highly and early upregulated during scrapie infections are ligands of cxcr3. to gain more insight into the role of cxcr3 in a prion model, cxcr3-deficient (cxcr3(-/-)) mice were infected intracerebrally with scrapie strain 139a and characterized in comparison to sim ... | 2008 | 18842729 |
| immunolocalisation of prpsc in scrapie-infected n2a mouse neuroblastoma cells by light and electron microscopy. | the causative agent of transmissible spongiform encephalopathies (tse) is prpsc, an infectious, misfolded isoform of the cellular prion protein (prpc). the localisation and trafficking of prpsc and sites of conversion from prpc to prpsc are under debate, particularly since most published work did not discriminate between prpc and prpsc. here we describe the localisation of prpc and prpsc in a scrapie-infected neuroblastoma cell line, scn2a, by light and electron microscopic immunolocalisation. a ... | 2009 | 18834644 |
| faecal shedding, alimentary clearance and intestinal spread of prions in hamsters fed with scrapie. | shedding of prions via faeces may be involved in the transmission of contagious prion diseases. here, we fed hamsters 10mg of 263k scrapie brain homogenate and examined the faecal excretion of disease-associated prion protein (prp(tse)) during the course of infection. the intestinal fate of ingested prp(tse) was further investigated by monitoring the deposition of the protein in components of the gut wall using immunohistochemistry and paraffin-embedded tissue (pet) blotting. western blotting of ... | 2009 | 18828985 |
| opposing effects of erk and p38-jnk map kinase pathways on formation of prions in gt1-1 cells. | brain-derived neurotrophic factor, which activates the extracellular regulated kinase (erk) pathway, increases formation of prions in scrapie-infected gonadotropin-releasing hormone (gt1-1) cells. this indicates that conversion of the cellular prion protein prp(c) to its pathogenic isoform, prp(sc), can be regulated by physiological stimuli acting on specific signal transduction pathways. in the present study, we examined the involvement of different mitogen-activated protein (map) kinase cascad ... | 2009 | 18824519 |
| production of prnp -/- goats by gene targeting in adult fibroblasts. | homozygous mice devoid of functional prnp are resistant to scrapie and prion propagation, but heterozygous mice for prnp disruption still suffer from prion disease and prion deposition. we have previously generated heterozygous cloned goats with one allele of prnp functional disruption. to obtain goats with both alleles of prnp be disrupted which would be resistant to scrapie completely, a second-round gene targeting was applied to disrupt the wild type allele of prnp in the heterozygous goats. ... | 2009 | 18821027 |
| reduced translocation of nascent prion protein during er stress contributes to neurodegeneration. | during acute stress in the endoplasmic reticulum (er), mammalian prion protein (prp) is temporarily prevented from translocation into the er and instead routed directly for cytosolic degradation. this "pre-emptive" quality control (pqc) system benefits cells by minimizing prp aggregation in the secretory pathway during er stress. however, the potential toxicity of cytosolic prp raised the possibility that persistent pqc of prp contributes to neurodegeneration in prion diseases. here, we find evi ... | 2008 | 18804434 |
| environmentally-relevant forms of the prion protein. | scrapie and chronic wasting disease (cwd) are prion diseases of particular environmental concern as they are horizontally transmissible and can remain infectious after years in the environment. recent evidence suggests that the n-terminus of prpsc, the infectious conformation of the prion protein, plays an important role in the mechanism of sorption to soil particles. we hypothesize that, in a prion-infected animal carcass, a portion of the n-terminus of prpsc could be cleaved by proteinases in ... | 2008 | 18800532 |
| neuroanatomical distribution of abnormal prion protein in naturally occurring atypical scrapie cases in great britain. | scrapie belongs to a group of diseases known as the transmissible spongiform encephalopathies or prion diseases. two different categories of naturally occurring scrapie have been identified: classical scrapie, which was first recorded around 1750, and atypical scrapie or 'nor-98', which was first identified in norway in 1998. the molecular characteristics of atypical scrapie have been well defined, but detailed descriptions of the neuropathological phenotype are rare since the majority of cases ... | 2008 | 18797889 |
| the effects of prion protein proteolysis and disaggregation on the strain properties of hamster scrapie. | native mammalian prions exist in self-propagating strains that exhibit distinctive clinical, pathological and biochemical characteristics. prion strain diversity is associated with variations in prp(sc) conformation, but it remains unknown precisely which physical properties of the prp(sc) molecules are required to encipher mammalian prion strain phenotypes. in this study, we subjected prion-infected brain homogenates derived from three different hamster scrapie strains to either (i) proteinase ... | 2008 | 18796735 |
| docosahexaenoic and eicosapentaenoic acids increase prion formation in neuronal cells. | the transmissible spongiform encephalopathies, otherwise known as prion diseases, occur following the conversion of the cellular prion protein (prpc) to an alternatively folded, disease-associated isoform (prpsc). recent studies suggest that this conversion occurs via a cholesterol-sensitive process, as cholesterol synthesis inhibitors reduced the formation of prpsc and delayed the clinical phase of scrapie infection. since polyunsaturated fatty acids also reduced cellular cholesterol levels we ... | 2008 | 18789130 |
| quantitative recovery of scrapie agent with minimal protein from highly infectious cultures. | there are few reports on the isolation, quantitative recovery, and relative purification of infectious particles that cause scrapie, creutzfeldt-jakob disease (cjd) and epidemic bovine spongiform encephalopathy (bse). because pure prion protein (prp) has failed to show significant infectivity, it is critical to find other molecules that are integral agent components. only complex diseased tissues such as degenerating brain have been fractionated, and agent recoveries have been quite low in conce ... | 2008 | 18788938 |
| accumulation of citrullinated proteins by up-regulated peptidylarginine deiminase 2 in brains of scrapie-infected mice: a possible role in pathogenesis. | peptidylarginine deiminases (pads), which are a group of posttranslational modification enzymes, are involved in protein citrullination (deimination) by the conversion of peptidylarginine to peptidylcitrulline in a calcium concentration-dependent manner. among the pads, pad2 is widely distributed in various tissues and is the only type that is expressed in brain. to elucidate the involvement of protein citrullination by pad2 in the pathogenesis of brain-specific prion diseases, we examined the p ... | 2008 | 18787103 |
| prp genotype frequencies of quebec sheep breeds determined by real-time pcr and molecular beacons. | the allele and genotype frequencies of the prion protein gene (prp), known to have an impact on scrapie susceptibility, were determined by real-time pcr for 500 quebec purebred rams. molecular beacons were very efficient in discriminating the 5 alleles investigated. polymorphisms at coding positions 136, 154, and 171 of the prp gene were analyzed using 3 separate real-time pcr reactions and a total of 7 molecular beacons. a total of 4 different alleles (arq, arr, ahr, and vrq) were observed at d ... | 2008 | 18783020 |
| [establishment of prp(sc) conversion based on serial pmca in vitro]. | in order to establish an amplification system in vitro with which the prp(sc) is able to convert prp(c) into proteinase k-resistant isoform infinitely and whether this system is more efficient than conventional protein misfolding cyclic amplification (pmca), scrapie strain 263k-infected hamster's brain homogenate and homologous normal brain homogenate were prepared, respectively. a new methodology, namely serial pmca, was utilized to reveal the continuous propagation ability of prp(sc). totally ... | 2008 | 18780631 |
| the effect of fenton reaction on protease-resistant prion protein (prpsc) degradation and scrapie infectivity. | in prion diseases, metal imbalances in brain and/or metal substitutions for copper in prion protein suggest that metal-catalyzed oxidation (mco) and oxidative stress may affect cellular function and accumulation of protease-resistant prion protein (prp(sc)). we examined the effect of metal-induced oxidative stress by fenton reaction on prion protein with regard to its degradation, insolubility, and infectivity. precipitation and insolubility of prion protein were induced by fenton reaction in sc ... | 2008 | 18771660 |
| a c-terminal protease-resistant prion fragment distinguishes ovine "ch1641-like" scrapie from bovine classical and l-type bse in ovine transgenic mice. | the protease-resistant prion protein (prp(res)) of a few natural scrapie isolates identified in sheep, reminiscent of the experimental isolate ch1641 derived from a british natural scrapie case, showed partial molecular similarities to ovine bovine spongiform encephalopathy (bse). recent discovery of an atypical form of bse in cattle, l-type bse or base, suggests that also this form of bse might have been transmitted to sheep. we studied by western blot the molecular features of prp(res) in four ... | 2008 | 18769714 |
| molecular and transmission characteristics of primary-passaged ovine scrapie isolates in conventional and ovine prp transgenic mice. | a more complete assessment of ovine prion strain diversity will be achieved by complementing biological strain typing in conventional and ovine prp transgenic mice with a biochemical analysis of the resultant prpsc. this will provide a correlation between ovine prion strain phenotype and the molecular nature of different prp conformers associated with particular prion strains. here, we have compared the molecular and transmission characteristics of ovine arq/arq and vrq/vrq scrapie isolates foll ... | 2008 | 18768980 |
| excretion of transmissible spongiform encephalopathy infectivity in urine. | the route of transmission of most naturally acquired transmissible spongiform encephalopathy (tse) infections remains speculative. to investigate urine as a potential source of tse exposure, we used a sensitive method for detection and quantitation of tse infectivity. pooled urine collected from 22 hamsters showing clinical signs of 263k scrapie contained 3.8 +/- 0.9 infectious doses/ml of infectivity. titration of homogenates of kidneys and urinary bladders from the same animals gave concentrat ... | 2008 | 18760007 |
| lipids in the assembly of membrane proteins and organization of protein supercomplexes: implications for lipid-linked disorders. | lipids play important roles in cellular dysfunction leading to disease. although a major role for phospholipids is in defining the membrane permeability barrier, phospholipids play a central role in a diverse range of cellular processes and therefore are important factors in cellular dysfunction and disease. this review is focused on the role of phospholipids in normal assembly and organization of the membrane proteins, multimeric protein complexes, and higher order supercomplexes. since lipids ... | 2008 | 18751913 |
| creb-dependent gene regulation by prion protein: impact on mmp-9 and beta-dystroglycan. | corruption of the normal function of the cellular prion protein (prp(c)) by the scrapie isoform (prp(sc)) emerges as a critical causal event in transmissible spongiform encaphalopathies (tse) pathogenesis. however, prp(c) physiological role remains unclear. by exploiting the properties of the 1c11 neuroectodermal cell line, able to convert into 1c11(5-ht) serotonergic or 1c11(ne) noradrenergic neuronal cells, we assigned a signaling function to prp(c). here, we establish that antibody-mediated p ... | 2008 | 18718863 |
| lactoferrin induces cell surface retention of prion protein and inhibits prion accumulation. | prion diseases are fatal neurodegenerative disorders, and the conformational conversion of normal cellular prion protein (prp(c)) into its pathogenic, amyloidogenic isoform (prp(sc)) is the essential event in the pathogenesis of these diseases. lactoferrin (lf) is a cationic iron-binding glycoprotein belonging to the transferrin (tf) family, which accumulates in the amyloid deposits in the brain in neurodegenerative disorders, such as alzheimer's disease and pick's disease. in the present study, ... | 2008 | 18717818 |
| involvement of glypican-1 autoprocessing in scrapie infection. | the copper-binding cellular prion protein (prp(c)) and the heparan sulphate (hs)-containing proteoglycan glypican-1 (gpc-1) can both be attached to lipid rafts via their glycosylphosphatidylinositol anchors, and copper ions stimulate their cointernalization from the cell surface to endosomes. the prion protein controls cointernalization and delivers copper necessary for s-nitrosylation of conserved cysteines in the gpc-1 core protein. later, during recycling through endosomal compartments, nitri ... | 2008 | 18717736 |
| investigation of mcp1 as a quantitative trait gene for prion disease incubation time in mouse. | the genetic basis of prion disease incubation time is principally determined by polymorphisms in the prion protein gene, prnp. however, it is now known that other genetic factors are important. several quantitative trait loci (qtl) have been identified across the genome including a broad region of linkage on mmu11. monocyte chemoattractant protein 1 (mcp-1) maps to this region and has been associated with microglial activation and reduced survival in the me7 mouse scrapie model of prion disease. ... | 2008 | 18716327 |
| accelerated prion disease pathogenesis in toll-like receptor 4 signaling-mutant mice. | prion diseases such as scrapie involve the accumulation of disease-specific prion protein, prp(sc), in the brain. toll-like receptors (tlrs) are a family of proteins that recognize microbial constituents and are central players in host innate immune responses. the tlr9 agonist unmethylated cpg dna was shown to prolong the scrapie incubation period in mice, suggesting that innate immune activation interferes with prion disease progression. thus, it was predicted that ablation of tlr signaling wou ... | 2008 | 18715916 |
| detection of infectious prions in urine. | prions are the infectious agents responsible for prion diseases, which appear to be composed exclusively by the misfolded prion protein (prp(sc)). the mechanism of prion transmission is unknown. in this study, we attempted to detect prions in urine of experimentally infected animals. prp(sc) was detected in approximately 80% of the animals studied, whereas no false positives were observed among the control animals. semi-quantitative calculations suggest that prp(sc) concentration in urine is aro ... | 2008 | 18706416 |
| increased oxidation, glycoxidation, and lipoxidation of brain proteins in prion disease. | the basic molecular underpinnings of the pathological changes that unfold in prion disease remain elusive. a key role of increased oxidative stress has been hypothesized. given the transient nature of most intermediate molecules implicated, increased oxidative stress is better assessed by quantitating the damage it causes to macromolecules. we used mass spectrometry-based methods to measure specific products of protein oxidation, glycoxidation, and lipoxidation in brains from patients suffering ... | 2008 | 18703134 |
| prion infected meat-and-bone meal is still infectious after biodiesel production. | the epidemic of bovine spongiform encephalopathy (bse) has led to a world-wide drop in the market for beef by-products, such as meat-and-bone meal (mbm), a fat-containing but mainly proteinaceaous product traditionally used as an animal feed supplement. while normal rendering is insufficient, the production of biodiesel from mbm has been suggested to destroy infectivity from transmissible spongiform encephalopathies (tses). in addition to producing fuel, this method simultaneously generates a nu ... | 2008 | 18698417 |
| prion gene (prnp) haplotype variation in united states goat breeds (open access publication). | scrapie eradication efforts cost 18 million dollars annually in the united states and rely heavily upon prnp genotyping of sheep. genetic resistance might reduce goat scrapie and limit the risk of goats serving as a scrapie reservoir, so prnp coding sequences were examined from 446 goats of 10 breeds, 8 of which had not been previously examined at prnp. the 10 observed alleles were all related to one of two central haplotypes by a single amino acid substitution. at least five of these alleles (m ... | 2008 | 18694550 |
| acute cellular uptake of abnormal prion protein is cell type and scrapie-strain independent. | transmissible spongiform encephalopathies (tses) are fatal neurodegenerative diseases that include creutzfeldt-jakob disease, bovine spongiform encephalopathy and sheep scrapie. although one of the earliest events during tse infection is the cellular uptake of protease resistant prion protein (prp-res), this process is poorly understood due to the difficulty of clearly distinguishing input prp-res from either prp-res or protease-sensitive prp (prp-sen) made by the cell. using prp-res tagged with ... | 2008 | 18692214 |
| effects of new amphotericin analogues on the scrapie isoform of the prion protein. | prion diseases or transmissible spongiform encephalopathies (tses) are a group of neurodegenerative disorders associated with the conversion of a normal host prion protein (prp(c)) into a pathogenic isoform (prp(sc)). despite years of research, there is still no known cure for tses. amphotericin b (amb), an anti-fungal antibiotic, has antiprion activity but its usage is limited by its toxicity. this study assessed the antiprion properties of new amphotericin analogues in which the exocyclic carb ... | 2008 | 18691635 |
| green tea extracts interfere with the stress-protective activity of prp and the formation of prp. | a hallmark in prion diseases is the conformational transition of the cellular prion protein (prp(c)) into a pathogenic conformation, designated scrapie prion protein (prp(sc)), which is the essential constituent of infectious prions. here, we show that epigallocatechin gallate (egcg) and gallocatechin gallate, the main polyphenols in green tea, induce the transition of mature prp(c) into a detergent-insoluble conformation distinct from prp(sc). the prp conformer induced by egcg was rapidly inter ... | 2008 | 18691383 |
| control of scrapie in the uk sheep population. | scrapie is a fatal transmissible spongiform encephalopathy (tse) of sheep, endemic in the uk for centuries. interest in the disease has been heightened over the last decade by the possibility of the related bse being transmissible to and between sheep and a range of control interventions has been proposed and implemented. in this paper, we examined the effect of these policies and their components on observed case rate, susceptible allele frequency and r0 within the framework of a large simulati ... | 2009 | 18687157 |
| transmission dynamics and mechanisms of endemicity of scrapie in the uk sheep population. | summaryscrapie is a fatal neurological disease of sheep which is endemic in the united kingdom. it is one of the family of transmissible spongiform encephalopathies (tses) that includes bse. in this paper, we developed a micro-simulation model for scrapie in the uk sheep population, incorporating the genetic and structural diversity of the population and infectious contact between flocks through trading. the simulation was fitted to epidemiological data from a range of sources. we found a detect ... | 2009 | 18687155 |
| small-ruminant lentivirus enhances prpsc accumulation in cultured sheep microglial cells. | sheep scrapie is the prototypical transmissible spongiform encephalopathy (prion disease), which has a fundamental pathogenesis involving conversion of normal cellular prion protein (prp(c) [c superscript stands for cellular]) to disease-associated prion protein (prp(sc) [sc superscript stands for sheep scrapie]). sheep microglial cell cultures, derived from a prnp 136vv/171qq near-term fetal brain, were developed to study sheep scrapie in the natural host and to investigate potential cofactors ... | 2008 | 18684809 |
| [establishment of an assay for prp(sc) detection based on streptomycin precipitation]. | to establish a new western blotting assay for prp(sc) detection, we optimized the western blotting assay with a precipitation procedure of streptomycin sulfate. after digestion with pk, 10% scrapie infected hamster brain homogenates were incubated with 60 mmol/l streptomycin and the precipitated prp(sc) was recovered by centrifugation. the enrichment of prp(sc) by streptomycin sulfate precipitation was evaluated using western blotting assay. the results showed streptomycin could bind to pk-treat ... | 2008 | 18683554 |
| breeding for scrapie resistance in the hungarian sheep population. | the first results of the hungarian sheep prion protein (prp) genotyping programme are discussed in this paper. to obtain initial genotype frequency data 10 commercial (hungarian merino, german mutton merino, merino landschaf, german blackheaded, suffolk, texel, ile de france, charollais, lacaune, british milksheep) and 4 indigenous (gyimes racka, hortobágy racka, tsigaja, cikta) breeds were sampled in 2003 and 2004, and the prp genotypes were determined by microsequencing analysis with capillary ... | 2008 | 18669244 |
| scrapie-induced defects in learning and memory of transgenic mice expressing anchorless prion protein are associated with alterations in the gamma aminobutyric acid-ergic pathway. | after infection with rml murine scrapie agent, transgenic (tg) mice expressing prion protein (prp) without its glycophosphatidylinositol (gpi) membrane anchor (gpi(-/-) prp tg mice) continue to make abundant amounts of the abnormally folded disease-associated prpres but have a normal life span. in contrast, all age-, sex-, and genetically matched mice with a gpi-anchored prp become moribund and die due to a chronic progressive neurodegenerative disease by 160 days after rml scrapie agent infecti ... | 2008 | 18667494 |
| synthetic fibril peptide promotes clearance of scrapie prion protein by lysosomal degradation. | transmissible spongiform encephalopathies are infectious and neurodegenerative disorders that cause neural deposition of aggregates of the disease-associated form of prp(sc). prp(sc) reproduces by recruiting and converting the cellular prp(c), and scn2a cells support prp(sc) propagation. we found that incubation of scn2a cells with a fibril peptide named p9, which comprises an intrinsic sequence of residues 167-184 of mouse prp(c), significantly reduced the amount of prp(sc) in 24 hr. p9 did not ... | 2008 | 18667034 |
| effect of the dimethoate administration on a scrapie murine model. | some authors have associated organophosphate compounds with susceptibility to transmissible spongiform encephalopathy (tse) and even with the origin of this group of diseases. nevertheless, the actual role played by these compounds still remains unclear. the aim of this study was to assess the effect of oral exposure to dimethoate (dmt) on the development of scrapie using a genetically modified murine model. a total of 70 c57bl/6 mice over-expressing the prp gene (tg20) were included in the pres ... | 2008 | 18667030 |
| extending zelterman's approach for robust estimation of population size to zero-truncated clustered data. | estimation of population size with missing zero-class is an important problem that is encountered in epidemiological assessment studies. fitting a poisson model to the observed data by the method of maximum likelihood and estimation of the population size based on this fit is an approach that has been widely used for this purpose. in practice, however, the poisson assumption is seldom satisfied. zelterman (1988) has proposed a robust estimator for unclustered data that works well in a wide class ... | 2008 | 18663764 |
| prion infection of mice transgenic for human appswe: increased accumulation of cortical formic acid extractable abeta(1-42) and rapid scrapie disease development. | neuropathological, epidemiological and experimental data indicate a potential interrelationship between alzheimer's disease and prion diseases. proteolytic processing of amyloid precursor protein (app) by beta-secretase was recently suggested to be controlled by prion protein expression. here, we characterized the prion infection of tg2576 mice, which overexpress the human app(swe) protein. prion infection of tg2576-mice led to an early death of the animals, which was preceded by a relatively sh ... | 2008 | 18662767 |
| characterisation of new monoclonal antibodies reacting with prions from both human and animal brain tissues. | post-mortem diagnosis of transmissible spongiform encephalopathies (prion diseases) is primarily based on the detection of a protease resistant, misfolded disease associated isoform (prp(sc)) of the prion protein (prp(c)) on neuronal cells. these methods depend on antibodies directed against prp(c) and capable of reacting with prp(sc)in situ (immunohistochemistry on nervous tissue sections) or with the unfolded form of the protein (western and paraffin embedded tissue (pet) blotting). here, high ... | 2008 | 18657541 |
| prion protein amino acid determinants of differential susceptibility and molecular feature of prion strains in mice and voles. | the bank vole is a rodent susceptible to different prion strains from humans and various animal species. we analyzed the transmission features of different prions in a panel of seven rodent species which showed various degrees of phylogenetic affinity and specific prion protein (prp) sequence divergences in order to investigate the basis of vole susceptibility in comparison to other rodent models. at first, we found a differential susceptibility of bank and field voles compared to c57bl/6 and wo ... | 2008 | 18654630 |
| prion diseases are efficiently transmitted by blood transfusion in sheep. | the emergence of variant creutzfeld-jakob disease, following on from the bovine spongiform encephalopathy (bse) epidemic, led to concerns about the potential risk of iatrogenic transmission of disease by blood transfusion and the introduction of costly control measures to protect blood supplies. we previously reported preliminary data demonstrating the transmission of bse and natural scrapie by blood transfusion in sheep. the final results of this experiment, reported here, give unexpectedly hig ... | 2008 | 18647958 |
| a gamma-secretase inhibitor and quinacrine reduce prions and prevent dendritic degeneration in murine brains. | in prion-infected mice, both the notch-1 intracellular domain transcription factor (nicd) and the disease-causing prion protein (prp(sc)) increase in the brain preceding dendritic atrophy and loss. because the drug ly411575 inhibits the gamma-secretase-catalyzed cleavage of notch-1 that produces nicd, we asked whether this gamma-secretase inhibitor (gsi) might prevent dendritic degeneration in mice with scrapie. at 50 d postinoculation with rocky mountain laboratory (rml) prions, mice were given ... | 2008 | 18647832 |
| delivery of single-chain antibodies (scfvs) directed against the 37/67 kda laminin receptor into mice via recombinant adeno-associated viral vectors for prion disease gene therapy. | the 37/67 kda laminin receptor (lrp/lr) acts as a receptor for prions providing a promising target for the treatment of prion diseases. recently, we selected anti-lrp/lr single-chain antibodies (scfvs) and proved a reduction of the peripheral prp(sc) propagation by passive immunotransfer into scrapie-infected mice. here, we report the development of an in vivo gene delivery system based on adeno-associated virus (aav) vectors expressing scfvs-s18 and -n3 directed against lrp/lr. transduction of ... | 2008 | 18632978 |
| scrapie resistance in arq sheep. | variation in the ovine prion protein amino acid sequence influences scrapie progression, with sheep homozygous for a(136)r(154)q(171) considered susceptible. this study examined the association of survival time of scrapie-exposed arq sheep with variation elsewhere in the ovine prion gene. four single nucleotide polymorphism alleles were associated with prolonged survival. one nonsynonymous allele (t112) was associated with an additional 687 days of survival for scrapie-exposed sheep compared to ... | 2008 | 18632863 |
| changes in protein structure and distribution observed at pre-clinical stages of scrapie pathogenesis. | scrapie is a neurodegenerative disorder that involves the misfolding, aggregation and accumulation of the prion protein (prp). the normal cellular prp (prp(c)) is rich in alpha-helical secondary structure, whereas the disease-associated pathogenic form of the protein (prp(sc)) has an anomalously high beta-sheet content. in this study, protein structural changes were examined in situ in the dorsal root ganglia from perorally 263k scrapie-infected and mock-infected hamsters using synchrotron fouri ... | 2008 | 18625306 |
| scrapie prion protein structural constraints obtained by limited proteolysis and mass spectrometry. | elucidation of the structure of scrapie prion protein (prp(sc)), essential to understand the molecular mechanism of prion transmission, continues to be one of the major challenges in prion research and is hampered by the insolubility and polymeric character of prp(sc). limited proteolysis is a useful tool to obtain insight on structural features of proteins: proteolytic enzymes cleave proteins more readily at exposed sites, preferentially within loops, and rarely in beta-strands. we treated prp( ... | 2008 | 18621059 |
| western blot detection of prp sc in archived paraffin-embedded brainstem from scrapie-affected sheep. | scrapie is a naturally occurring fatal neurodegenerative disease of adult sheep and goats, one of a group of mammalian diseases known as transmissible spongiform encephalopathies (tse) or prion diseases. immunoassays that identify disease-associated prion protein (prp sc) are integral to the diagnosis of scrapie and other prion diseases. results obtained by either immunohistochemistry (ihc) or western blot (wb) assay are generally adequate for the definitive diagnosis. approved or accepted metho ... | 2008 | 18599864 |
| [scrapie of sheep and creutzfeldt-jakob disease in iceland]. | scrapie of sheep and creutzfeldt-jakob disease (cjd) are both classified as prion diseases. the infectious agents of both diseases are closely related. the objectives of the study was to explore, whether sheep scrapie could be transmitted to humans and cause cjd. | 2008 | 18591729 |
| mouse-adapted sporadic human creutzfeldt-jakob disease prions propagate in cell culture. | cell based models used for the study of prion diseases have traditionally employed mouse-adapted strains of sheep scrapie prions. to date, attempts to generate human prion propagation in cell culture have been unsuccessful. rabbit kidney epithelial cells (rk13) are permissive to infection with prions from a variety of species upon expression of cognate prp transgenes. we explored rk13 cells expressing human prp for their utility as a cell line capable of sustaining infection with human prions. r ... | 2008 | 18590830 |
| prion disease causes less severe lesions in human hippocampus than other parts of brain. | the hippocampus can be very sensitive to damage in the scrapie-infected mouse, a well-established animal model of prion diseases. terminally ill scrapie-infected animals exhibit nearly complete loss of cornu ammonis (ca) 1 pyramidal neurons, but few studies have focused on the neuropathological lesions of the human hippocampus in autopsied brain tissue; in particular, few findings on differences in severity of pathology between the hippocampal and parahippocampal formations have been obtained. t ... | 2008 | 18588585 |
| a histopathologic and immunohistochemical review of archived uk caprine scrapie cases. | in 2005, a prion disease identified in a goat from france was reported to be consistent with disease from the bovine spongiform encephalopathy (bse) agent. subsequent retrospective examination of uk goat scrapie cases led to the identification of one potentially similar, but as yet unconfirmed, case from scotland. these findings strengthened concerns that small ruminant populations exposed to the bse agent have become infected. the lack of data relating specifically to scrapie in goats has been ... | 2008 | 18587090 |
| risks of transmitting ruminant spongiform encephalopathies (prion diseases) by semen and embryo transfer techniques. | early experiments suggested that scrapie transmission via sheep embryos was a possibility, and gave rise to much controversy. however, when account is taken of the complex genetic effects on ovine susceptibility to scrapie, and of the several different scrapie strains with different clinical and pathological effects, the overall conclusion now is that transmission of classical scrapie by embryo transfer is very unlikely if appropriate precautions are taken. recent embryo transfer studies have co ... | 2008 | 18586320 |
| spontaneous and bse-prion-seeded amyloid formation of full length recombinant bovine prion protein. | the conversion of the cellular isoform of the prion protein into the pathogenic isoform prp(sc) is the key event in prion diseases. the disease can occur spontaneously genetically or by infection. in earlier studies we presented an in vitro conversion system which simulates the structural transition in recprp by varying low concentrations of sds at constant nacl. in the present study we adopted the conversion system from experimental scrapie in hamster to bovine recprp and generated amyloid fibr ... | 2008 | 18585368 |
| temporary depletion of cd11c+ dendritic cells delays lymphoinvasion after intraperitonal scrapie infection. | the involvement of immune cells in prion capture and transport to lymphoid tissues still remains unclear. to investigate the role of dendritic cells (dc), we used dtr(+/+) mice, a transgenic model designed to trigger short-term ablation of dc. transient depletion of dc around the time of intraperitoneal infection delayed prion replication in the spleen, as followed by prpsc amount, a specific hallmark of prion diseases. consequently, neuroinvasion and incubation time of prion disease were delaye ... | 2008 | 18579603 |
| assaying prions in cell culture: the standard scrapie cell assay (ssca) and the scrapie cell assay in end point format (scepa). | prions are usually quantified by bioassays based on intracerebral inoculation of animals, which are slow, imprecise, and costly. we have developed a cell-based prion assay that is based on the isolation of cell lines highly susceptible to certain strains (rocky mountain laboratory and 22l) of mouse prions and a method for identifying individual, prion-infected cells and quantifying them. in the standard scrapie cell assay (ssca), susceptible cells are exposed to prion-containing samples for 4 da ... | 2008 | 18576147 |
| cell culture models to unravel prion protein function and aberrancies in prion diseases. | from an early stage of prion research, tissue cultures that could support and propagate the scrapie agent were sought after. the earliest attempts were explants from brains of infected mice, and their growth and morphological characteristics were compared with those from uninfected mice. using the explant technique, several investigators reported increased cell growth in cultures established from scrapie-sick brain compared with cultures from normal mice. these are odd findings in the light of t ... | 2008 | 18576144 |
| glutathione peroxidase (gpx) activity in blood of ewes on farms in different scrapie categories in iceland. | preliminary studies indicated decreased glutathione peroxidase (gpx) activity in blood of ewes on scrapie-afflicted farms. other studies have shown decreased gpx activity in brain of prion-infected mice and in prion-infected cells in vitro. the aim of this study was to examine the gpx activity in blood as well as the distribution of gpx-activity levels from ewes on farms in scrapie-afflicted areas in iceland. | 2008 | 18573202 |
| the in vitro bioassay systems for the amplification and detection of abnormal prion prp(sc) in blood and tissues. | prion diseases or transmissible spongiform encephalopathies, including creutzfeldt-jakob disease (cjd) and variant cjd (vcjd), are chronic neurodegenerative diseases characterized by accumulation of abnormal infectious prions known as prpsc. infectious prion is emerging as a blood transfusion-transmissible pathogen and is present at extremely low levels in the blood of asymptomatic patients, which is not detectable by current standard methodologies. as such, prion-related diseases impose a huge ... | 2008 | 18572098 |
| stress-protective signalling of prion protein is corrupted by scrapie prions. | studies in transgenic mice revealed that neurodegeneration induced by scrapie prion (prp(sc)) propagation is dependent on neuronal expression of the cellular prion protein prp(c). on the other hand, there is evidence that prp(c) itself has a stress-protective activity. here, we show that the toxic activity of prp(sc) and the protective activity of prp(c) are interconnected. with a novel co-cultivation assay, we demonstrate that prp(sc) can induce apoptotic signalling in prp(c)-expressing cells. ... | 2008 | 18566584 |
| contribution of antibody and t cell-specific responses to the progression of 139a-scrapie in c57bl/6 mice immunized with prion protein peptides. | prion diseases are associated with the conversion of the normal host cellular prion protein to an abnormal protease-resistant (prpres) associated with infectivity. no specific immune response against prions develops during infection due to the strong tolerance to cellular prion protein. we examined the protective potential on prion diseases of immune responses elicited in c57bl/6 mice with prp peptides 98-127 (p5) or 158-187 (p9) with cpg. after immunization, p5-treated mice developed high titer ... | 2008 | 18566443 |
| antiprion activity of functionalized 9-aminoacridines related to quinacrine. | a library of functionalized 6-chloro-2-methoxy-(n(9)-substituted)acridin-9-amines structurally related to quinacrine were synthesized and evaluated for antiprion activity on four different cell models persistently infected with scrapie prion strains (scn2a, n167, ch2) or a human disease prion strain (f3). most of the compounds were distinguished by the side chain attached to 9-amino of the acridine ring. these were dialkylaminoalkyl and phenyl with basic groups on the phenyl ring. the most promi ... | 2008 | 18556207 |
| effect of scrapie incubation on the concentrations of plasma amino acids and l-lactate in infected lambs. | three groups of two weeks old growing lambs differing in prp genotype were orally inoculated with scrapie and maintained under defined conditions until disease endpoint. plasma concentrations of free alanine and serine, but not l-lactate increased during the final 6 months of the disease. at the same time, plasma concentrations of several essential and non-essential free amino acids decreased linearly, indicating reduced feed intake and are consistent with, but occurring before establishment, of ... | 2008 | 18548327 |
| a descriptive study of the prevalence of atypical and classical scrapie in sheep in 20 european countries. | the development of active surveillance programmes for transmissible spongiform encephalopathies of small ruminants across europe has led to the recent identification of a previously undetected form of ovine prion disease, 'atypical' scrapie. knowledge of the epidemiology of this disease is still limited, as is whether it represents a risk for animal and/or public health. the detection of atypical scrapie has been related to the use of only some of the eu agreed rapid tests. information about the ... | 2008 | 18544152 |
| determination of sex and scrapie resistance genotype in preimplantation ovine embryos. | the aim of this study was to test the accuracy of genotype diagnosis after pre-amplification of dna extracted from biopsies obtained by microblade cutting of ovine embryos and to evaluate the viability of biopsied embryos after vitrification/warming and transfer to recipients. sex and prp genotypes were determined. sex diagnosis was done by pcr amplification of zfx/zfy and sry sequences after pep-pcr while prp genotype determination was performed after specific pre-amplification of specific targ ... | 2009 | 18543282 |
| induced neuroprotection independently from prpsc accumulation in a mouse model for prion disease treated with simvastatin. | the misfolding and aggregation of specific proteins has emerged as a key feature of several neurodegenerative diseases. in prion diseases, progressive disease and neuronal loss are associated with the accumulation of prp(sc), the misfolded isoform of prp(c). previous in vitro studies suggest that cholesterol-lowering drugs inhibit the conversion of prp(c) to prp(sc) and the accumulation of the latter, possibly through the disturbance of cholesterol-rich membrane domains (lipid rafts). | 2008 | 18541796 |
| polymorphisms of the prion protein gene in sheep of inner mongolia, china. | polymorphisms of the prion protein gene (prnp), especially the amino acid residue alterations at codons 136, 154, and 174, in sheep have been found to be associated with susceptibility to scrapie disease. we investigated prnp polymorphisms in three local sheep breeds in inner mongolia, china. blood samples were collected from 46 ujumqin, 34 sunite, and 22 mongolian sheep. the genetic dna of blood samples was extracted, amplified and sequenced, and amino acid alignment was determined. polymorphis ... | 2008 | 18521732 |
| atypical scrapie in a sheep in a closed uk flock with endemic classical natural scrapie. | 2008 | 18515761 | |
| transmission and detection of prions in feces. | in chronic wasting disease (cwd) in cervids and in scrapie in sheep, prions appear to be transmitted horizontally. oral exposure to prion-tainted blood, urine, saliva, and feces has been suggested as the mode of transmission of cwd and scrapie among herbivores susceptible to these prion diseases. to explore the transmission of prions through feces, uninoculated syrian hamsters (shas) were cohabitated with or exposed to the bedding of shas orally infected with sc237 prions. incubation times of 14 ... | 2008 | 18505383 |
| antimicrobial use in the alberta sheep industry. | information regarding antimicrobial use in sheep is scarce. in 2001, a scrapie surveillance program was initiated in alberta that also provided a mechanism for collecting other sheep health data including antimicrobial use information between april 2001 and april 2002. a major objective of this study was to describe antimicrobial use in the alberta sheep industry. this was done by obtaining qualitative antimicrobial use information from all flocks (n = 212) providing cull ewes to the program usi ... | 2008 | 18505202 |
| identification of new quantitative trait loci (other than the prnp gene) modulating the scrapie incubation period in sheep. | although susceptibility to scrapie is largely controlled by the prnp gene, we have searched for additional genomic regions that affect scrapie incubation time in sheep, using two half-sib families with a susceptible prnp genotype and naturally infected by scrapie. quantitative trait loci were detected on oar6 and oar18. | 2008 | 18493086 |
| prion early kinetics revisited using a streptomycin-based prp(res) extraction method. | the use of streptomycin in the prp(sc) detection procedures represents a new and attractive way to detect more prp(sc), the best marker for the transmissible spongiform encephalopathies (tses). actually, the streptomycin prp(sc) aggregating property reported recently was established as beneficial in prp(sc) detection using immunohistochemistry in diagnostic as well as in experimental conditions. the present study reports in details how to use advantageously this original streptomycin property in ... | 2008 | 18489903 |
| transmissible spongiform encephalopathy strain-associated diversity of n-terminal proteinase k cleavage sites of prp(sc) from scrapie-infected and bovine spongiform encephalopathy-infected mice. | assessment of the different conformational states of the abnormal prion protein (prp(sc)) in the cns provides an established basis for distinguishing transmissible spongiform encephalopathy (tse) strains. prp(sc) conformers are variably resistant to n-terminal proteinase k (pk) digestion, and analysis of the consensus products (prp(res)) by immunoassay enables effective, but relatively low-resolution differentiation. determination of the precise n-terminal amino acid profile (n-taap) of prp(res) ... | 2008 | 18484354 |
| virus-induced alterations of membrane lipids affect the incorporation of prp sc into cells. | prion diseases are fatal neurodegenerative disorders characterized by long incubation periods. to investigate whether concurrent diseases can modify the clinical outcome of prion-affected subjects, we tested the effect of viral infection on the binding and internalization of prp(sc), essential steps of prion propagation. to this effect, we added scrapie brain homogenate or purified prp(sc) to fibroblasts previously infected with minute virus of mice (mvm), a mouse parvovirus. we show here that t ... | 2008 | 18478553 |
| prion propagation in mice lacking central nervous system nf-kappab signalling. | prions induce highly typical histopathological changes including cell death, spongiosis and activation of glia, yet the molecular pathways leading to neurodegeneration remain elusive. following prion infection, enhanced nuclear factor-kappab (nf-kappab) activity in the brain parallels the first pathological changes. the nf-kappab pathway is essential for proliferation, regulation of apoptosis and immune responses involving induction of inflammation. the ikappab kinase (ikk) signalosome is crucia ... | 2008 | 18474572 |
| effect of intraventricular infusion of anti-prion protein monoclonal antibodies on disease progression in prion-infected mice. | it is well known that anti-prion protein (prp) monoclonal antibodies (mabs) inhibit abnormal isoform prp (prpsc) formation in cell culture. additionally, passive immunization of anti-prp mabs protects the animals from prion infection via peripheral challenge when mabs are administered simultaneously or soon after prion inoculation. thus, anti-prp mabs are candidates for the treatment of prion diseases. however, the effects of mabs on disease progression in the middle and late stages of the disea ... | 2008 | 18474571 |
| cholesterol transporter atp-binding cassette a1 (abca1) is elevated in prion disease and affects prpc and prpsc concentrations in cultured cells. | prion diseases are transmissible neurodegenerative disorders of prion protein (prp) conformation. prion replication by conversion of benign prpc isoforms into disease-specific prpsc isoforms is intimately involved in prion disease pathogenesis and may be initiated in cholesterol-rich caveolae-like domains (cld). concentrations of the cholesterol transporter atp-binding cassette a1 protein (abca1) are elevated in pre-clinical scrapie prion-infected mice and in prion-infected cells in vitro. eleva ... | 2008 | 18474570 |
| prion diseases and emerging prion diseases. | transmissible spongiform encephalopathies (tses), also called prion diseases, are fatal neurodegenerative disorders. an abnormal isoform of the prion protein (prp(sc)) generated by post-translational modification of the cellular prion protein (prp(c)) is believed to be the main component of this infectious agent. prp(sc) is relatively resistant to proteinase k (pk) digestion. this characteristic has been widely accepted as the physicochemical basis for distinguishing between prp(c) and prp(sc). ... | 2008 | 18473798 |
| the key-role of tyrosine 155 in the mechanism of prion transconformation as highlighted by a study of sheep mutant peptides. | prion protein is a strongly conserved and ubiquitous glycoprotein. the conformational conversion of the non-pathogenic cellular prion isoform (prp(c)) into a pathogenic scrapie isoform (prp(sc)) is a fundamental event in the onset of transmissible spongiform encephalopathies (tse). during this conversion, helix h1 and its two flanking loops are known to undergo a conformational transition into a beta-like structure. in order to understand mechanisms which trigger this transconformation, sheep pr ... | 2008 | 18455265 |
| pruritus is a common feature in sheep infected with the bse agent. | the variability in the clinical or pathological presentation of transmissible spongiform encephalopathies (tses) in sheep, such as scrapie and bovine spongiform encephalopathy (bse), has been attributed to prion protein genotype, strain, breed, clinical duration, dose, route and type of inoculum and the age at infection. the study aimed to describe the clinical signs in sheep infected with the bse agent throughout its clinical course to determine whether the clinical signs were as variable as de ... | 2008 | 18445253 |
| increase of monoamine oxidase-b activity in the brain of scrapie-infected hamsters. | in the present study, the purpose is to determine activities of monoamine oxidases (mao) in the brain of 263k scrapie-infected hamsters during the development of this experimental prion disease. indeed, mao activity modifications which have already been related in aging and neurodegenerations is suspected to be involved in the neuron loss process by elevated hydrogen peroxide formation. monoamine oxidase type a (mao-a) and b (mao-b) activities were followed in the brain at different stages of th ... | 2008 | 18442871 |
| prominent pancreatic endocrinopathy and altered control of food intake disrupt energy homeostasis in prion diseases. | prion diseases are fatal neurodegenerative diseases that can induce endocrinopathies. the basis of altered endocrine function in prion diseases is not well understood, and the purpose of this study was to investigate the spatiotemporal relationship between energy homeostasis and prion infection in hamsters inoculated with either the 139h strain of scrapie agent, which induces preclinical weight gain, or the hy strain of transmissible mink encephalopathy (tme), which induces clinical weight loss. ... | 2008 | 18434355 |
| spatial correlation between the prevalence of transmissible spongiform diseases and british soil geochemistry. | transmissible spongiform encephalopathies (tses) are a group of fatal neurological conditions affecting a number of mammals, including sheep and goats (scrapie), cows (bse), and humans (creutzfeldt-jakob disease). the diseases are widely believed to be caused by the misfolding of the normal prion protein to a pathological isoform, which is thought to act as an infectious agent. outbreaks of the disease are commonly attributed to contaminated feed and genetic susceptibility. however, the implicat ... | 2009 | 18427934 |
| myenteric neurons of the ileum that express somatostatin are a target of prion neuroinvasion in an alimentary model of sheep scrapie. | neuroinvasion of the enteric nervous system by prions is an important step in dissemination to the brain, yet very little is known about the basic process of enteric neuroinvasion. using an alimentary model of neonatal disease transmission, neuroinvasion by scrapie prions in the ileum of lambs was detected by immunohistochemical staining for the disease-associated form of the prion protein, prpsc. odds ratios (or) were determined for the frequency of prpsc staining within enteric somata categori ... | 2008 | 18427817 |
| scrapie transmission via milk. | 2008 | 18424852 | |
| atypical scrapie in a sheep in scotland. | 2008 | 18424849 | |
| canine mdck cell lines are refractory to infection with human and mouse prions. | influenza vaccine production in embryonated eggs is associated with many disadvantages, and production in cell culture systems is a viable alternative. madin darby canine kidney (mdck) cells are permissive for a variety of orthomyxoviruses and have proven particularly suitable for vaccine mass production. however, mammalian cells harboring the prnp gene can theoretically acquire prion infections. here, we have attempted to infect mdck cells and substrains thereof with prions. we found that mdck ... | 2008 | 18423803 |