prpsc proteins

Summary

Summary: Abnormal isoform of PRION PROTEINS resulting from a posttranslational modification of the cellular prion protein (PRPC PROTEINS). PrPSc are disease-specific proteins seen in certain human and animal neurodegenerative diseases (PRION DISEASES).

Top Publications

  1. Gonzalez Montalban N, Makarava N, Ostapchenko V, Savtchenk R, Alexeeva I, Rohwer R, et al. Highly efficient protein misfolding cyclic amplification. PLoS Pathog. 2011;7:e1001277 pubmed publisher
    ..The new PMCAb format offers immediate practical benefits and opens new avenues for developing fast ultrasensitive assays and for producing abundant quantities of PrP(Sc)in vitro...
  2. Rubenstein R, Bulgin M, Chang B, Sorensen Melson S, Petersen R, LaFauci G. PrP(Sc) detection and infectivity in semen from scrapie-infected sheep. J Gen Virol. 2012;93:1375-83 pubmed publisher
    ..These results suggest that the transmissible agent, or at least the seeding activity, for sheep scrapie is present in semen. This may be a strain-specific phenomenon. ..
  3. Goold R, McKinnon C, Rabbanian S, Collinge J, Schiavo G, Tabrizi S. Alternative fates of newly formed PrPSc upon prion conversion on the plasma membrane. J Cell Sci. 2013;126:3552-62 pubmed publisher
    ..This pathway leads to lysosomal degradation, and we show that this is the dominant PrP(Sc) degradative mechanism in the early stages of prion infection. ..
  4. Yam A, Gao C, Wang X, Wu P, Peretz D. The octarepeat region of the prion protein is conformationally altered in PrP(Sc). PLoS ONE. 2010;5:e9316 pubmed publisher
    ..This phenomenon may be relevant to the mechanism by which the amino terminus of PrP(C) participates in PrP(Sc) conversion, and may also be exploited for diagnostic purposes. ..
  5. Beck K, Sallis R, Lockey R, Simmons M, Spiropoulos J. Ovine PrP genotype is linked with lesion profile and immunohistochemistry patterns after primary transmission of classical scrapie to wild-type mice. J Neuropathol Exp Neurol. 2010;69:483-97 pubmed publisher
    ..These results indicate that ovine PrP genotype is a factor that is linked to both the lesion profile and the pattern of PrP deposition on primary transmission of classical scrapie to wild-type mice. ..
  6. Jen A, Parkyn C, Mootoosamy R, Ford M, Warley A, Liu Q, et al. Neuronal low-density lipoprotein receptor-related protein 1 binds and endocytoses prion fibrils via receptor cluster 4. J Cell Sci. 2010;123:246-55 pubmed publisher
  7. Ushiki Kaku Y, Endo R, Iwamaru Y, Shimizu Y, Imamura M, Masujin K, et al. Tracing conformational transition of abnormal prion proteins during interspecies transmission by using novel antibodies. J Biol Chem. 2010;285:11931-6 pubmed publisher
    ..Such mAbs capable of discriminating conformational differences may allow us to address questions concerning PrP(Sc) conformation and strain diversity. ..
  8. Edwards J, Moore S, Hawthorn J, Neale M, Terry L. PrP(Sc) is associated with B cells in the blood of scrapie-infected sheep. Virology. 2010;405:110-9 pubmed publisher
    ..Furthermore, tests for preclinical diagnostics could be further improved by targeting these cells. ..
  9. Corda E, Beck K, Sallis R, Vickery C, Denyer M, Webb P, et al. The interpretation of disease phenotypes to identify TSE strains in mice: characterisation of BSE using PrPSc distribution patterns in the brain. Vet Res. 2012;43:86 pubmed publisher
    ..The detailed description presented in this study provides a reference document for identifying BSE in wild type mice. ..

More Information

Publications106 found, 100 shown here

  1. Ono F, Terao K, Tase N, Hiyaoka A, Ohyama A, Tezuka Y, et al. Experimental transmission of bovine spongiform encephalopathy (BSE) to cynomolgus macaques, a non-human primate. Jpn J Infect Dis. 2011;64:50-4 pubmed
    ..Neither IHC nor WB detected PrP(Sc) in the lymphatic organs/tissues, such as the tonsils, spleen, and appendix. ..
  2. Monaco S, Fiorini M, Farinazzo A, Ferrari S, Gelati M, Piccardo P, et al. Allelic origin of protease-sensitive and protease-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease with the P102L mutation. PLoS ONE. 2012;7:e32382 pubmed publisher
  3. O Rourke K, Schneider D, Spraker T, Dassanayake R, Highland M, Zhuang D, et al. Transmissibility of caprine scrapie in ovine transgenic mice. BMC Vet Res. 2012;8:42 pubmed publisher
  4. Thackray A, Hopkins L, Lockey R, Spiropoulos J, Bujdoso R. Propagation of ovine prions from "poor" transmitter scrapie isolates in ovine PrP transgenic mice. Exp Mol Pathol. 2012;92:167-74 pubmed publisher
    ..These novel observations in ovine PrP transgenic mice highlight a new approach to ovine prion strain typing. ..
  5. Hara H, Okemoto Nakamura Y, Shinkai Ouchi F, Hanada K, Yamakawa Y, Hagiwara K. Mouse prion protein (PrP) segment 100 to 104 regulates conversion of PrP(C) to PrP(Sc) in prion-infected neuroblastoma cells. J Virol. 2012;86:5626-36 pubmed publisher
    ..These results suggest the segment 100 to 104 of MoPrP(C) plays a key role in conversion after binding to MoPrP(Sc). ..
  6. Sarasa R, Martinez A, Monleón E, Bolea R, Vargas A, Badiola J, et al. Involvement of astrocytes in transmissible spongiform encephalopathies: a confocal microscopy study. Cell Tissue Res. 2012;350:127-34 pubmed publisher
    ..Furthermore, despite ongoing debate regarding whether varied TSE sources show differences in their tropism for different cell lineages in the brains of affected animals, no differences in co-localization results were seen. ..
  7. Okada H, Iwamaru Y, Fukuda S, Yokoyama T, Mohri S. Detection of disease-associated prion protein in the optic nerve and the adrenal gland of cattle with bovine spongiform encephalopathy by using highly sensitive immunolabeling procedures. J Histochem Cytochem. 2012;60:290-300 pubmed publisher
    ..This study also suggests that tyramide-based immunochemical analysis should be performed to detect immunolabeled PrP(Sc) in the extracerebral tissues of BSE-affected cattle...
  8. Bett C, Joshi Barr S, Lucero M, Trejo M, Liberski P, Kelly J, et al. Biochemical properties of highly neuroinvasive prion strains. PLoS Pathog. 2012;8:e1002522 pubmed publisher
  9. Kim J, Cali I, Surewicz K, Kong Q, Raymond G, Atarashi R, et al. Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors. J Biol Chem. 2010;285:14083-7 pubmed publisher
  10. Wang H, Wan J, Wang W, Wang D, Li S, Liao P, et al. Overexpression of Shadoo protein in transgenic mice does not impact the pathogenesis of scrapie. Neurosci Lett. 2011;496:1-4 pubmed publisher
    ..We therefore conclude that Shadoo has little or no influence on the outcome of transmissible spongiform encephalopathy (TSE) disease in transgenic mice. ..
  11. Miyazawa K, Emmerling K, Manuelidis L. High CJD infectivity remains after prion protein is destroyed. J Cell Biochem. 2011;112:3630-7 pubmed publisher
    ..Our FU-CJD brain results are in good accord with the only other report on maximal PrP destruction and titer. It is likely that one or more residual non-PrP proteins may protect agent nucleic acids in infectious particles. ..
  12. Kim C, Haldiman T, Surewicz K, Cohen Y, Chen W, Blevins J, et al. Small protease sensitive oligomers of PrPSc in distinct human prions determine conversion rate of PrP(C). PLoS Pathog. 2012;8:e1002835 pubmed publisher
  13. McGovern G, Mabbott N, Jeffrey M. Scrapie affects the maturation cycle and immune complex trapping by follicular dendritic cells in mice. PLoS ONE. 2009;4:e8186 pubmed publisher
    ..We therefore support the hypothesis that TSEs cause an abnormality in immune function. ..
  14. Tabouret G, Lacroux C, Lugan S, Costes P, Corbière F, Weisbecker J, et al. Relevance of oral experimental challenge with classical scrapie in sheep. J Gen Virol. 2010;91:2139-44 pubmed publisher
    ..Nevertheless, data obtained under such experimental conditions should be used with some caution. ..
  15. Spiropoulos J, Lockey R, Sallis R, Terry L, Thorne L, Holder T, et al. Isolation of prion with BSE properties from farmed goat. Emerg Infect Dis. 2011;17:2253-61 pubmed publisher
    ..Surveillance should continue so that another outbreak of this zoonotic transmissible spongiform encephalopathy can be prevented and public health safeguarded...
  16. Morales R, Estrada L, Diaz Espinoza R, Morales Scheihing D, Jara M, Castilla J, et al. Molecular cross talk between misfolded proteins in animal models of Alzheimer's and prion diseases. J Neurosci. 2010;30:4528-35 pubmed publisher
    ..Our findings may have important implications to understand the origin and progression of PMDs. ..
  17. Fischer M, Appelhans D, Schwarz S, Klajnert B, Bryszewska M, Voit B, et al. Influence of surface functionality of poly(propylene imine) dendrimers on protease resistance and propagation of the scrapie prion protein. Biomacromolecules. 2010;11:1314-25 pubmed publisher
  18. Cosseddu G, Nonno R, Vaccari G, Bucalossi C, Fernandez Borges N, Di Bari M, et al. Ultra-efficient PrP(Sc) amplification highlights potentialities and pitfalls of PMCA technology. PLoS Pathog. 2011;7:e1002370 pubmed publisher
    ..Our findings underline the potential drawback of ultrasensitive in vitro prion replication and warn on cautious interpretation when assessing the spontaneous appearance of prions in vitro. ..
  19. Tribouillard Tanvier D, Striebel J, Peterson K, Chesebro B. Analysis of protein levels of 24 cytokines in scrapie agent-infected brain and glial cell cultures from mice differing in prion protein expression levels. J Virol. 2009;83:11244-53 pubmed publisher
  20. Deleault N, Kascsak R, Geoghegan J, Supattapone S. Species-dependent differences in cofactor utilization for formation of the protease-resistant prion protein in vitro. Biochemistry. 2010;49:3928-34 pubmed publisher
    ..These results reveal species-specific differences in cofactor utilization for PrP(Sc) propagation in vitro and also demonstrate the existence of an endogenous cofactor present in brain tissue not composed of nucleic acids. ..
  21. Mazza M, Iulini B, Vaccari G, Acutis P, Martucci F, Esposito E, et al. Co-existence of classical scrapie and Nor98 in a sheep from an Italian outbreak. Res Vet Sci. 2010;88:478-85 pubmed publisher
  22. McCutcheon S, Alejo Blanco A, Houston E, de Wolf C, Tan B, Smith A, et al. All clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vCJD. PLoS ONE. 2011;6:e23169 pubmed publisher
    ..These data suggest that all blood components are vectors for prion disease transmission, and highlight the importance of multiple control measures to minimise the risk of human to human transmission of vCJD by blood transfusion. ..
  23. Barash J. Clinical features of sporadic fatal insomnia. Rev Neurol Dis. 2009;6:E87-93 pubmed
    ..SFI may also present like other neurodegenerative diseases, and common diagnostic findings that are seen in other forms of sporadic CJD may be absent. ..
  24. Bate C, Tayebi M, Williams A. Glycosylphosphatidylinositol anchor analogues sequester cholesterol and reduce prion formation. J Biol Chem. 2010;285:22017-26 pubmed publisher
    ..We propose that treatment with glucosamine-PI modifies local micro-environments that control PrP(C) expression and activation of PLA(2) and subsequently inhibits PrP(Sc) formation. ..
  25. Vrentas C, Greenlee J, Tatum T, Nicholson E. Relationships between PrPSc stability and incubation time for United States scrapie isolates in a natural host system. PLoS ONE. 2012;7:e43060 pubmed publisher
  26. Wang F, Wang X, Ma J. Conversion of bacterially expressed recombinant prion protein. Methods. 2011;53:208-13 pubmed publisher
    ..The establishment of the in vitro recombinant PrP conversion assay makes it feasible for us to explore the molecular basis behind the intriguing properties associated with prion infectivity. ..
  27. Balkema Buschmann A, Ziegler U, McIntyre L, Keller M, Hoffmann C, Rogers R, et al. Experimental challenge of cattle with German atypical bovine spongiform encephalopathy (BSE) isolates. J Toxicol Environ Health A. 2011;74:103-9 pubmed publisher
    ..Eleven calves were thus challenged intracranially, five with the German H-type and six with German L-type BSE cases. The experimental design and the clinical studies, followed by laboratory testing, are described in this manuscript. ..
  28. Gough K, Maddison B. Prion transmission: prion excretion and occurrence in the environment. Prion. 2010;4:275-82 pubmed
  29. Makarava N, Kovacs G, Savtchenko R, Alexeeva I, Budka H, Rohwer R, et al. Genesis of mammalian prions: from non-infectious amyloid fibrils to a transmissible prion disease. PLoS Pathog. 2011;7:e1002419 pubmed publisher
    ..The current work provides important new insight into the mechanisms underlying genesis of the transmissible protein states and has numerous implications for understanding the etiology of neurodegenerative diseases. ..
  30. Okada H, Sakurai M, Yokoyama T, Mohri S. Disease-associated prion protein in the dental tissue of mice infected with scrapie. J Comp Pathol. 2010;143:218-22 pubmed publisher
    ..Dental tissue might therefore be a potential source of PrP(Sc) for horizontal transmission of TSEs. ..
  31. Beck K, Vickery C, Lockey R, Holder T, Thorne L, Terry L, et al. The interpretation of disease phenotypes to identify TSE strains following murine bioassay: characterisation of classical scrapie. Vet Res. 2012;43:77 pubmed publisher
    ..These data provide a comprehensive description of classical scrapie strain phenotypes on isolation through the mouse bioassay that can provide a reference for further scrapie strain identification. ..
  32. Kim J, Surewicz K, Gambetti P, Surewicz W. The role of glycophosphatidylinositol anchor in the amplification of the scrapie isoform of prion protein in vitro. FEBS Lett. 2009;583:3671-5 pubmed publisher
    ..These findings shed a new light on prion protein conversion process and have important implications for the efforts to generate synthetic prions for structural and biophysical studies. ..
  33. Hedlin P, Cashman N, Li L, Gupta J, Babiuk L, Potter A, et al. Design and delivery of a cryptic PrP(C) epitope for induction of PrP(Sc)-specific antibody responses. Vaccine. 2010;28:981-8 pubmed publisher
    ..Antigen specific antibodies are also present within cerebral spinal fluid and mucosal secretions. These characteristics provide a foundation for development of a TSE vaccine. ..
  34. Shikiya R, Ayers J, Schutt C, Kincaid A, Bartz J. Coinfecting prion strains compete for a limiting cellular resource. J Virol. 2010;84:5706-14 pubmed publisher
    ..This was confirmed by PMCA detection of HY PrP(Sc) in animals where DY TME had completely blocked HY TME from causing disease...
  35. Ano Y, Sakudo A, Uraki R, Sato Y, Kono J, Sugiura K, et al. Enhanced enteric invasion of scrapie agents into the villous columnar epithelium via maternal immunoglobulin. Int J Mol Med. 2010;26:845-51 pubmed
    ..In the present study, it was suggested that the weaning period and maternal immunoglobulin are important risk factors for the oral transmission of PrPSc. ..
  36. Gilch S, Bach C, Lutzny G, Vorberg I, Schatzl H. Inhibition of cholesterol recycling impairs cellular PrP(Sc) propagation. Cell Mol Life Sci. 2009;66:3979-91 pubmed publisher
    ..Surprisingly, rab 9 overexpression itself reduced cellular PrP(Sc) content, indicating that PrP(Sc) production is highly sensitive to alterations in dynamics of vesicle trafficking. ..
  37. Li J, Browning S, Mahal S, Oelschlegel A, Weissmann C. Darwinian evolution of prions in cell culture. Science. 2010;327:869-72 pubmed publisher
    ..Prions, albeit devoid of a nucleic acid genome, are thus subject to mutation and selective amplification. ..
  38. Cancellotti E, Bradford B, Tuzi N, Hickey R, Brown D, Brown K, et al. Glycosylation of PrPC determines timing of neuroinvasion and targeting in the brain following transmissible spongiform encephalopathy infection by a peripheral route. J Virol. 2010;84:3464-75 pubmed publisher
    ..This demonstrates that targeting in the brain following peripheral inoculation is profoundly influenced by the glycosylation status of host PrP. ..
  39. Monleón E, Garza M, Sarasa R, Alvarez Rodriguez J, Bolea R, Monzon M, et al. An assessment of the efficiency of PrPsc detection in rectal mucosa and third-eyelid biopsies from animals infected with scrapie. Vet Microbiol. 2011;147:237-43 pubmed publisher
  40. D Castro L, Wenborn A, Gros N, Joiner S, Cronier S, Collinge J, et al. Isolation of proteinase K-sensitive prions using pronase E and phosphotungstic acid. PLoS ONE. 2010;5:e15679 pubmed publisher
    ..This procedure now allows characterization of proteinase K-sensitive prions and investigation of their clinical relevance in human and animal prion disease without being confounded by contaminating PrP(C)...
  41. Gong B, Ramos A, Vázquez Fernández E, Silva C, Alonso J, Liu Z, et al. Probing structural differences between PrP(C) and PrP(Sc) by surface nitration and acetylation: evidence of conformational change in the C-terminus. Biochemistry. 2011;50:4963-72 pubmed publisher
    ..3) The amino-terminal region of SHaPrP 27-30 is very accessible. These data should help in the validation and construction of structural models of PrP(Sc)...
  42. Ishibashi D, Atarashi R, Fuse T, Nakagaki T, Yamaguchi N, Satoh K, et al. Protective role of interferon regulatory factor 3-mediated signaling against prion infection. J Virol. 2012;86:4947-55 pubmed publisher
    ..Our findings provide new insight into the role of the host innate immune system in the pathogenesis of prion diseases. ..
  43. Masujin K, Shu Y, Okada H, Matsuura Y, Iwamaru Y, Imamura M, et al. Isolation of two distinct prion strains from a scrapie-affected sheep. Arch Virol. 2009;154:1929-32 pubmed publisher
    ..In subsequent passages, the mice showed distinct characteristic scrapie phenotypes. This finding may prove that different prion strains coexist in a naturally affected sheep with scrapie. ..
  44. Rubenstein R, Chang B, Gray P, Piltch M, Bulgin M, Sorensen Melson S, et al. A novel method for preclinical detection of PrPSc in blood. J Gen Virol. 2010;91:1883-92 pubmed publisher
    ..We did not find any enhancement of sPMCA with the addition of polyadenylic acid nor was it necessary to match the genotypes of the PrP(C) and PrP(Sc) sources for efficient amplification. ..
  45. Pirisinu L, Di Bari M, Marcon S, Vaccari G, D Agostino C, Fazzi P, et al. A new method for the characterization of strain-specific conformational stability of protease-sensitive and protease-resistant PrPSc. PLoS ONE. 2010;5:e12723 pubmed publisher
  46. Maddison B, Owen J, Bishop K, Shaw G, Rees H, Gough K. The interaction of ruminant PrP(Sc) with soils is influenced by prion source and soil type. Environ Sci Technol. 2010;44:8503-8 pubmed publisher
    ..These data indicate that natural ruminant prion strains are stable in the soil environment for at least 18 months and that PrP(Sc)-soil interaction is dictated by both the soil properties and the strain/host species of PrP(Sc). ..
  47. Orru C, Caughey B. Prion seeded conversion and amplification assays. Top Curr Chem. 2011;305:121-33 pubmed publisher
    ..This chapter will provide an overview of such reactions and the practical and fundamental consequences of their development. ..
  48. Deriziotis P, Andre R, Smith D, Goold R, Kinghorn K, Kristiansen M, et al. Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry. EMBO J. 2011;30:3065-77 pubmed publisher
    ..A similar inhibition of substrate entry into the proteasome may occur in other neurodegenerative diseases where misfolded ?-sheet-rich proteins accumulate. ..
  49. Leblanc P, Hasenkrug K, Ward A, Myers L, Messer R, Alais S, et al. Co-infection with the friend retrovirus and mouse scrapie does not alter prion disease pathogenesis in susceptible mice. PLoS ONE. 2012;7:e30872 pubmed publisher
    ..Thus, retroviral co-infection does not necessarily alter prion disease pathogenesis in vivo, most likely because of different cell-specific sites of replication for scrapie and F-MuLV. ..
  50. Andreoletti O, Litaise C, Simmons H, Corbière F, Lugan S, Costes P, et al. Highly efficient prion transmission by blood transfusion. PLoS Pathog. 2012;8:e1002782 pubmed publisher
    ..These results demonstrate that TSE transmission by blood transfusion can be highly efficient and that this efficiency is more dependent on the viability of transfused cells than the level of infectivity measured by IC inoculation. ..
  51. Parchi P, Strammiello R, Notari S, Giese A, Langeveld J, Ladogana A, et al. Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification. Acta Neuropathol. 2009;118:659-71 pubmed publisher
    ..The present data provide an updated disease classification and are of importance for future epidemiologic and transmission studies aimed to identify etiology and extent of strain variation in sporadic Creutzfeldt-Jakob disease. ..
  52. Neale M, Mountjoy S, Edwards J, Vilette D, Laude H, Windl O, et al. Infection of cell lines with experimental and natural ovine scrapie agents. J Virol. 2010;84:2444-52 pubmed publisher
    ..Increasing the sensitivity of the Rov9 cell line to SSBP/1 infection did not correlate with broadening susceptibility, as the specificity of permissiveness and resistance to other scrapie isolates was maintained. ..
  53. Ghaemmaghami S, May B, Renslo A, Prusiner S. Discovery of 2-aminothiazoles as potent antiprion compounds. J Virol. 2010;84:3408-12 pubmed publisher
    ..Our results establish 2-aminothiazoles as promising candidates for efficacy studies of animals and validate our drug discovery platform as a viable strategy for the identification of novel lead compounds with antiprion properties. ..
  54. Bishop M, Will R, Manson J. Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci U S A. 2010;107:12005-10 pubmed publisher
    ..Moreover, we have identified four distinct strains of sCJD using our in vivo strain typing panel. ..
  55. Mays C, Ryou C. Plasminogen stimulates propagation of protease-resistant prion protein in vitro. FASEB J. 2010;24:5102-12 pubmed publisher
    ..Our results demonstrate that plasminogen functions in stimulating conversion processes and represents the first cellular protein cofactor that enhances the hypothetical mechanism of prion propagation. ..
  56. Bate C, Williams A. Monoacylated cellular prion protein modifies cell membranes, inhibits cell signaling, and reduces prion formation. J Biol Chem. 2011;286:8752-8 pubmed publisher
    ..In addition, our observations raise the possibility that pharmacological modification of GPI anchors might constitute a novel therapeutic approach to prion diseases. ..
  57. Hoffmann C, Eiden M, Kaatz M, Keller M, Ziegler U, Rogers R, et al. BSE infectivity in jejunum, ileum and ileocaecal junction of incubating cattle. Vet Res. 2011;42:21 pubmed publisher
    ..The systematic approach of this study provides new data for qualitative and quantitative risk assessments and allows defining bovine SRM more precisely...
  58. TAMGUNEY G, Miller M, Wolfe L, Sirochman T, Glidden D, Palmer C, et al. Asymptomatic deer excrete infectious prions in faeces. Nature. 2009;461:529-32 pubmed publisher
  59. Wang F, Wang X, Yuan C, Ma J. Generating a prion with bacterially expressed recombinant prion protein. Science. 2010;327:1132-5 pubmed publisher
    ..Thus, as postulated by the prion hypothesis, the infectivity in mammalian prion disease results from an altered conformation of PrP. ..
  60. Dron M, Moudjou M, Chapuis J, Salamat M, Bernard J, Cronier S, et al. Endogenous proteolytic cleavage of disease-associated prion protein to produce C2 fragments is strongly cell- and tissue-dependent. J Biol Chem. 2010;285:10252-64 pubmed publisher
    ..These findings have implications on the molecular analysis of PrP(Sc) and cell pathogenesis of prion infection. ..
  61. Bessen R, Shearin H, Martinka S, Boharski R, Lowe D, Wilham J, et al. Prion shedding from olfactory neurons into nasal secretions. PLoS Pathog. 2010;6:e1000837 pubmed publisher
  62. Dudas S, Yang J, Graham C, Czub M, McAllister T, Coulthart M, et al. Molecular, biochemical and genetic characteristics of BSE in Canada. PLoS ONE. 2010;5:e10638 pubmed publisher
    ..The occurrence of atypical cases of BSE in countries such as Canada with low BSE prevalence and transmission risk argues for the occurrence of sporadic forms of BSE worldwide. ..
  63. Colby D, Prusiner S. Prions. Cold Spring Harb Perspect Biol. 2011;3:a006833 pubmed publisher
    ..1993). The conformational conversion of PrP(C) to PrP(Sc) is the fundamental event underlying prion diseases. In this article, we provide an introduction to prions and the diseases they cause. ..
  64. Godsave S, Wille H, Pierson J, Prusiner S, Peters P. Plasma membrane invaginations containing clusters of full-length PrPSc are an early form of prion-associated neuropathology in vivo. Neurobiol Aging. 2013;34:1621-31 pubmed publisher
    ..Only a small proportion of clustered PrP immunogold labeling was found at synapses, indicating that synapses are not targeted specifically in prion disease. ..
  65. Horiuchi M, Karino A, Furuoka H, Ishiguro N, Kimura K, Shinagawa M. Generation of monoclonal antibody that distinguishes PrPSc from PrPC and neutralizes prion infectivity. Virology. 2009;394:200-7 pubmed publisher
    ..Taken together, these results suggest that mAb 6H10 recognizes a conformational epitope on PrP(Sc) that is related to prion infectivity. ..
  66. Silva J, Gomes M, Vieira T, Cordeiro Y. PrP interactions with nucleic acids and glycosaminoglycans in function and disease. Front Biosci (Landmark Ed). 2010;15:132-50 pubmed
    ..These molecules seem to play a dual role in prion biology and could be fundamental to explaining how prion diseases arise, as well as in the development of effective therapeutic approaches. ..
  67. Mitchell G, O Rourke K, Harrington N, Soutyrine A, Simmons M, Dudas S, et al. Identification of atypical scrapie in Canadian sheep. J Vet Diagn Invest. 2010;22:408-11 pubmed
    ..Enhanced scrapie surveillance efforts recently identified 3 cases of atypical scrapie in Canada. ..
  68. Chen B, Morales R, Barria M, Soto C. Estimating prion concentration in fluids and tissues by quantitative PMCA. Nat Methods. 2010;7:519-20 pubmed publisher
    ..Using this procedure, we determined the quantities of misfolded prion protein (PrP(Sc)) in brain, spleen, blood and urine of scrapie-affected hamsters...
  69. Gill A, Agarwal S, Pinheiro T, Graham J. Structural requirements for efficient prion protein conversion: cofactors may promote a conversion-competent structure for PrP(C). Prion. 2010;4:235-42 pubmed publisher
    ..It is suggested that one role for molecular cofactors in the conversion process is to stabilize PrP(C) structure in a form that is amenable for conversion to PrP(Sc). ..
  70. Kim C, Haldiman T, Cohen Y, Chen W, Blevins J, Sy M, et al. Protease-sensitive conformers in broad spectrum of distinct PrPSc structures in sporadic Creutzfeldt-Jakob disease are indicator of progression rate. PLoS Pathog. 2011;7:e1002242 pubmed publisher
    ..The link between disease duration, levels, and stability of protease-sensitive conformers of PrP(Sc) suggests that these conformers play an important role in the pathogenesis of sCJD. ..
  71. Breyer J, Wemheuer W, Wrede A, Graham C, Benestad S, Brenig B, et al. Detergents modify proteinase K resistance of PrP Sc in different transmissible spongiform encephalopathies (TSEs). Vet Microbiol. 2012;157:23-31 pubmed publisher
    ..A plausible explanation for the detergent effects described in this article could be an interaction with the lipids associated with PrP(Sc) that may stabilize the aggregates. ..
  72. Diaz Espinoza R, Soto C. High-resolution structure of infectious prion protein: the final frontier. Nat Struct Mol Biol. 2012;19:370-7 pubmed publisher
    ..This review highlights the importance of, challenges for and recent progress toward elucidating the elusive structure of PrP(Sc), arguably the major pending milestone to reach in understanding prions. ..
  73. Kaatz M, Fast C, Ziegler U, Balkema Buschmann A, Hammerschmidt B, Keller M, et al. Spread of classic BSE prions from the gut via the peripheral nervous system to the brain. Am J Pathol. 2012;181:515-24 pubmed publisher
    ..In summary, our results decipher the centripetal spread of BSE prions along the autonomic nervous system to the central nervous system, starting already halfway in the incubation time...
  74. Diaz Espinoza R, Soto C. Generation of prions in vitro and the protein-only hypothesis. Prion. 2010;4:53-9 pubmed
    ..In this article, we discuss these recent reports under the context of the protein-only hypothesis and their implications. ..
  75. Jeffrey M, McGovern G, Sisó S, Gonzalez L. Cellular and sub-cellular pathology of animal prion diseases: relationship between morphological changes, accumulation of abnormal prion protein and clinical disease. Acta Neuropathol. 2011;121:113-34 pubmed publisher
    ..Thus, the proximate cause of clinical disease in animal prion disease is uncertain, but may not involve PrP(d). ..
  76. Eiden M, Hoffmann C, Balkema Buschmann A, Muller M, Baumgartner K, Groschup M. Biochemical and immunohistochemical characterization of feline spongiform encephalopathy in a German captive cheetah. J Gen Virol. 2010;91:2874-83 pubmed publisher
    ..The widespread PrP(Sc) deposition suggests a simultaneous lymphatic and neural spread of the FSE agent. The detection of PrP(Sc) in the spleen indicates a potential for prion infectivity of cheetah blood. ..
  77. Okada H, Sato Y, Sata T, Sakurai M, Endo J, Yokoyama T, et al. Antigen retrieval using sodium hydroxide for prion immunohistochemistry in bovine spongiform encephalopathy and scrapie. J Comp Pathol. 2011;144:251-6 pubmed publisher
    ..1% Triton-X at room temperature for 10 min and 150 mM NaOH at 60 °C for 10 min. By this simple procedure, PrP(Sc) was visualized in the brain of animals with BSE and scrapie using a range of anti-PrP primary antibodies...
  78. Vulin J, Biacabe A, Cazeau G, Calavas D, Baron T. Molecular typing of protease-resistant prion protein in transmissible spongiform encephalopathies of small ruminants, France, 2002-2009. Emerg Infect Dis. 2011;17:55-63 pubmed publisher
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    ..This feature, combined with the N-terminal PrPSc cleavage, allowed differentiation of classical scrapie, including CH1641-like, from natural goat BSE and experimental sheep BSE. ..
  80. Terry L, Howells L, Bishop K, Baker C, Everest S, Thorne L, et al. Detection of prions in the faeces of sheep naturally infected with classical scrapie. Vet Res. 2011;42:65 pubmed publisher
    ..These data represent the first demonstration of prion shedding in faeces from a naturally infected host and thus a likely source of prion contamination in the environment. ..
  81. Gough K, Baker C, Rees H, Terry L, Spiropoulos J, Thorne L, et al. The oral secretion of infectious scrapie prions occurs in preclinical sheep with a range of PRNP genotypes. J Virol. 2012;86:566-71 pubmed publisher
    ..The significance of positive sPMCA reactions was confirmed by the transmission of infectivity in buccal swab extracts to Tg338 mice, illustrating the likely importance of orally available prions in the horizontal transmission of scrapie...
  82. Oumata N, Nguyen P, Beringue V, Soubigou F, Pang Y, Desban N, et al. The toll-like receptor agonist imiquimod is active against prions. PLoS ONE. 2013;8:e72112 pubmed publisher
    ..Furthermore, we found that IQ and its active chemical derivatives inhibit the protein folding activity of the ribosome (PFAR) in vitro. ..
  83. Ghaemmaghami S, Ullman J, Ahn M, St Martin S, Prusiner S. Chemical induction of misfolded prion protein conformers in cell culture. J Biol Chem. 2010;285:10415-23 pubmed publisher
    ..It remains to be established whether the formation of PrP(A) inhibits the formation of rPrP(Sc) by sequestering PrP(C) in the form of benign, insoluble aggregates. ..
  84. Makarava N, Kovacs G, Bocharova O, Savtchenko R, Alexeeva I, Budka H, et al. Recombinant prion protein induces a new transmissible prion disease in wild-type animals. Acta Neuropathol. 2010;119:177-87 pubmed publisher
    ..Our studies establish that transmissible prion disease can be induced in wild-type animals by inoculation of rPrP and introduce a valuable new model of prion diseases...
  85. Tattum M, Jones S, Pal S, Collinge J, Jackson G. Discrimination between prion-infected and normal blood samples by protein misfolding cyclic amplification. Transfusion. 2010;50:996-1002 pubmed publisher
    ..The use of whole blood as an analyte circumvents the need to identify the optimal blood compartment for analysis and guarantees the totality of misfolded PrP will be available for detection. ..
  86. Yokoyama T, Masujin K, Schmerr M, Shu Y, Okada H, Iwamaru Y, et al. Intraspecies prion transmission results in selection of sheep scrapie strains. PLoS ONE. 2010;5:e15450 pubmed publisher
    ..Our results indicate that prion strain selection occurs after both inter- and intraspecies transmission. The unusual scrapie prion was a hidden or an unexpressed component in typical sheep scrapie...
  87. Sajnani G, Silva C, Ramos A, Pastrana M, Onisko B, Erickson M, et al. PK-sensitive PrP is infectious and shares basic structural features with PK-resistant PrP. PLoS Pathog. 2012;8:e1002547 pubmed publisher
    ..Although the sPrP(Sc) and rPrP(Sc) fractions have different resistance to PK-digestion, and have previously been shown to sediment differently, and have a different distribution of multimers, they share a common structure and phenotype. ..
  88. Peden A, McCardle L, Head M, Love S, Ward H, Cousens S, et al. Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia. Haemophilia. 2010;16:296-304 pubmed publisher
  89. Santucciu C, Maestrale C, Madau L, Attene S, Cancedda M, Demontis F, et al. Association of N176K and L141F dimorphisms of the PRNP gene with lack of pathological prion protein deposition in placentas of naturally and experimentally scrapie-affected ARQ/ARQ sheep. J Gen Virol. 2010;91:2402-7 pubmed publisher
    ..This finding demonstrates for the first time that fetal PRNP polymorphisms, other than those at codon 171, are associated with the lack of placental deposition of PrP(Sc)...
  90. Mahal S, Browning S, Li J, Suponitsky Kroyter I, Weissmann C. Transfer of a prion strain to different hosts leads to emergence of strain variants. Proc Natl Acad Sci U S A. 2010;107:22653-8 pubmed publisher
    ..Our results support the view that 22L prion populations are heterogeneous and that distinct prion variants are selected in different cellular environments. ..
  91. Wadsworth J, Dalmau Mena I, Joiner S, Linehan J, O Malley C, Powell C, et al. Effect of fixation on brain and lymphoreticular vCJD prions and bioassay of key positive specimens from a retrospective vCJD prevalence study. J Pathol. 2011;223:511-8 pubmed publisher
    ..In this context, the Hilton et al study should continue to inform risk assessment pending the outcome of larger-scale studies on discarded surgical tissues and autopsy samples...
  92. Andreoletti O, Orge L, Benestad S, Beringue V, Litaise C, Simon S, et al. Atypical/Nor98 scrapie infectivity in sheep peripheral tissues. PLoS Pathog. 2011;7:e1001285 pubmed publisher
    ..The results obtained indicate that dietary exposure risk to small ruminants TSE agents may be higher than commonly believed. ..