DeArmond SJ, Prusiner SB: Perspectives on prion biology, prion disease pathogenesis, and pharmacologic approaches to treatment. Clin Lab Med. 2003, 23 (1): 1-41.
Article
Google Scholar
Bossers A, Rigter A, de Vries R, Smits MA: In vitro conversion of normal prion protein into pathologic isoforms. Clin Lab Med. 2003, 23 (1): 227-247.
Article
Google Scholar
Govaerts C, Wille H, Prusiner SB, Cohen FE: Evidence for assembly of prions with left-handed beta-helices into trimers. Proc Natl Acad Sci USA. 2004, 101 (22): 8342-8347.
Article
PubMed Central
CAS
Google Scholar
Langedijk JP, Fuentes G, Boshuizen R, Bonvin AM: Two-rung Model of a Left-handed beta-Helix for Prions Explains Species Barrier and Strain Variation in Transmissible Spongiform Encephalopathies. J Mol Biol. 2006, 360 (4): 907-920.
Article
CAS
Google Scholar
Bossers A, de Vries R, Smits MA: Susceptibility of sheep for scrapie as assessed by in vitro conversion of nine naturally occurring variants of PrP. J Virol. 2000, 74 (3): 1407-1414.
Article
PubMed Central
CAS
Google Scholar
Kocisko DA, Priola SA, Raymond GJ, Chesebro B, Lansbury PT, Caughey B: Species specificity in the cell-free conversion of prion protein to protease-resistant forms: a model for the scrapie species barrier. Proc Natl Acad Sci USA. 1995, 92 (9): 3923-3927.
Article
PubMed Central
CAS
Google Scholar
Castilla J, Saa P, Morales R, Abid K, Maundrell K, Soto C: Protein misfolding cyclic amplification for diagnosis and prion propagation studies. Methods Enzymol. 2006, 412: 3-21.
Article
CAS
Google Scholar
Castilla J, Saa P, Soto C: Detection of prions in blood. Nat Med. 2005, 11 (9): 982-985.
CAS
Google Scholar
Saborio GP, Permanne B, Soto C: Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding. Nature. 2001, 411 (6839): 810-813.
Article
CAS
Google Scholar
Soto C, Anderes L, Suardi S, Cardone F, Castilla J, Frossard MJ, Peano S, Saa P, Limido L, Carbonatto M: Pre-symptomatic detection of prions by cyclic amplification of protein misfolding. FEBS Lett. 2005, 579 (3): 638-642.
Article
CAS
Google Scholar
Dubois MA, Sabatier P, Durand B, Calavas D, Ducrot C, Chalvet-Monfray K: Multiplicative genetic effects in scrapie disease susceptibility. C R Biol. 2002, 325 (5): 565-570.
Article
Google Scholar
Tranulis MA: Influence of the prion protein gene, Prnp, on scrapie susceptibility in sheep. Apmis. 2002, 110 (1): 33-43.
Article
CAS
Google Scholar
Sabuncu E, Petit S, Le Dur A, Lan Lai T, Vilotte JL, Laude H, Vilette D: PrP polymorphisms tightly control sheep prion replication in cultured cells. J Virol. 2003, 77 (4): 2696-2700.
Article
PubMed Central
CAS
Google Scholar
Rigter A, Bossers A: Sheep scrapie susceptibility-linked polymorphisms do not modulate the initial binding of cellular to disease-associated prion protein prior to conversion. J Gen Virol. 2005, 86 (Pt 9): 2627-2634.
Article
CAS
Google Scholar
Rigter A, Langeveld JP, Timmers-Parohi D, Jacobs JG, Moonen PL, Bossers A: Mapping of possible prion protein self interaction domains using peptide arrays. BMC Biochem. 2007, 8 (1): 6-
Article
PubMed Central
Google Scholar
Pan T, Wong BS, Liu T, Li R, Petersen RB, Sy MS: Cell-surface prion protein interacts with glycosaminoglycans. Biochem J. 2002, 368 (Pt 1): 81-90.
Article
PubMed Central
CAS
Google Scholar
Brown DR: Prion protein expression aids cellular uptake and veratridine-induced release of copper. J Neurosci Res. 1999, 58 (5): 717-725.
Article
CAS
Google Scholar
Brown DR: Role of the prion protein in copper turnover in astrocytes. Neurobiol Dis. 2004, 15 (3): 534-543.
Article
CAS
Google Scholar
Brown DR, Clive C, Haswell SJ: Antioxidant activity related to copper binding of native prion protein. J Neurochem. 2001, 76 (1): 69-76.
Article
CAS
Google Scholar
Dupuis L, Mbebi C, Gonzalez de Aguilar JL, Rene F, Muller A, de Tapia M, Loeffler JP: Loss of prion protein in a transgenic model of amyotrophic lateral sclerosis. Mol Cell Neurosci. 2002, 19 (2): 216-224.
Article
CAS
Google Scholar
Flechsig E, Shmerling D, Hegyi I, Raeber AJ, Fischer M, Cozzio A, von Mering C, Aguzzi A, Weissmann C: Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in PrP knockout mice. Neuron. 2000, 27 (2): 399-408.
Article
CAS
Google Scholar
Frankenfield KN, Powers ET, Kelly JW: Influence of the N-terminal domain on the aggregation properties of the prion protein. Protein Sci. 2005, 14 (8): 2154-2166.
Article
PubMed Central
CAS
Google Scholar
Klamt F, Dal-Pizzol F, Conte da Frota MJ, Walz R, Andrades ME, da Silva EG, Brentani RR, Izquierdo I, Fonseca Moreira JC: Imbalance of antioxidant defense in mice lacking cellular prion protein. Free Radic Biol Med. 2001, 30 (10): 1137-1144.
Article
CAS
Google Scholar
Miele G, Jeffrey M, Turnbull D, Manson J, Clinton M: Ablation of cellular prion protein expression affects mitochondrial numbers and morphology. Biochem Biophys Res Commun. 2002, 291 (2): 372-377.
Article
CAS
Google Scholar
Pauly PC, Harris DA: Copper stimulates endocytosis of the prion protein. J Biol Chem. 1998, 273 (50): 33107-33110.
Article
CAS
Google Scholar
Rachidi W, Vilette D, Guiraud P, Arlotto M, Riondel J, Laude H, Lehmann S, Favier A: Expression of prion protein increases cellular copper binding and antioxidant enzyme activities but not copper delivery. J Biol Chem. 2003, 278 (11): 9064-9072.
Article
CAS
Google Scholar
Sakudo A, Lee DC, Saeki K, Nakamura Y, Inoue K, Matsumoto Y, Itohara S, Onodera T: Impairment of superoxide dismutase activation by N-terminally truncated prion protein (PrP) in PrP-deficient neuronal cell line. Biochem Biophys Res Commun. 2003, 308 (3): 660-667.
Article
CAS
Google Scholar
Walz R, Amaral OB, Rockenbach IC, Roesler R, Izquierdo I, Cavalheiro EA, Martins VR, Brentani RR: Increased sensitivity to seizures in mice lacking cellular prion protein. Epilepsia. 1999, 40 (12): 1679-1682.
Article
CAS
Google Scholar
Wong BS, Pan T, Liu T, Li R, Gambetti P, Sy MS: Differential contribution of superoxide dismutase activity by prion protein in vivo. Biochem Biophys Res Commun. 2000, 273 (1): 136-139.
Article
CAS
Google Scholar
Wong E, Thackray AM, Bujdoso R: Copper induces increased beta-sheet content in the scrapie-susceptible ovine prion protein PrPVRQ compared with the resistant allelic variant PrPARR. Biochem J. 2004, 380 (Pt 1): 273-282.
Article
PubMed Central
CAS
Google Scholar
Zeng F, Watt NT, Walmsley AR, Hooper NM: Tethering the N-terminus of the prion protein compromises the cellular response to oxidative stress. J Neurochem. 2003, 84 (3): 480-490.
Article
CAS
Google Scholar
Jacobs JG, Langeveld JP, Biacabe AG, Acutis PL, Polak MP, Gavier-Widen D, Buschmann A, Caramelli M, Casalone C, Mazza M: Molecular discrimination of atypical bovine spongiform encephalopathy strains from a geographical region spanning a wide area in Europe. J Clin Microbiol. 2007, 45 (6): 1821-1829.
Article
PubMed Central
CAS
Google Scholar
Thuring CM, Erkens JH, Jacobs JG, Bossers A, Van Keulen LJ, Garssen GJ, Van Zijderveld FG, Ryder SJ, Groschup MH, Sweeney T: Discrimination between scrapie and bovine spongiform encephalopathy in sheep by molecular size, immunoreactivity, and glycoprofile of prion protein. J Clin Microbiol. 2004, 42 (3): 972-980.
Article
PubMed Central
CAS
Google Scholar
Knowles TP, Zahn R: Enhanced stability of human prion proteins with two disulfide bridges. Biophys J. 2006, 91 (4): 1494-1500.
Article
PubMed Central
CAS
Google Scholar
Korth C, Stierli B, Streit P, Moser M, Schaller O, Fischer R, Schulz-Schaeffer W, Kretzschmar H, Raeber A, Braun U: Prion (PrPSc)-specific epitope defined by a monoclonal antibody. Nature. 1997, 390 (6655): 74-77.
Article
CAS
Google Scholar
Kuczius T, Grassi J, Karch H, Groschup MH: Binding of N- and C-terminal anti-prion protein antibodies generates distinct phenotypes of cellular prion proteins (PrPC) obtained from human, sheep, cattle and mouse. Febs J. 2007, 274 (6): 1492-1502.
Article
CAS
Google Scholar
Martucci F, Acutis P, Mazza M, Nodari S, Colussi S, Corona C, Barocci S, Gabrielli A, Caramelli M, Casalone C: Detection of typical and atypical BSE and scrapie prion strains by prion protein motif-grafted antibodies. J Gen Virol. 2009
Google Scholar
Moroncini G, Kanu N, Solforosi L, Abalos G, Telling GC, Head M, Ironside J, Brockes JP, Burton DR, Williamson RA: Motif-grafted antibodies containing the replicative interface of cellular PrP are specific for PrPSc. Proc Natl Acad Sci USA. 2004, 101 (28): 10404-10409.
Article
PubMed Central
CAS
Google Scholar
Moroncini G, Mangieri M, Morbin M, Mazzoleni G, Ghetti B, Gabrielli A, Williamson RA, Giaccone G, Tagliavini F: Pathologic prion protein is specifically recognized in situ by a novel PrP conformational antibody. Neurobiol Dis. 2006, 23 (3): 717-724.
Article
CAS
Google Scholar
Paramithiotis E, Pinard M, Lawton T, LaBoissiere S, Leathers VL, Zou WQ, Estey LA, Lamontagne J, Lehto MT, Kondejewski LH: A prion protein epitope selective for the pathologically misfolded conformation. Nat Med. 2003, 9 (7): 893-899.
Article
CAS
Google Scholar
Horiuchi M, Baron GS, Xiong LW, Caughey B: Inhibition of interactions and interconversions of prion protein isoforms by peptide fragments from the C-terminal folded domain. J Biol Chem. 2001, 276 (18): 15489-15497.
Article
CAS
Google Scholar
Solforosi L, Bellon A, Schaller M, Cruite JT, Abalos GC, Williamson RA: Toward Molecular Dissection of PrPC-PrPSc Interactions. J Biol Chem. 2007, 282 (10): 7465-7471.
Article
CAS
Google Scholar
Soto C, Saborio GP, Anderes L: Cyclic amplification of protein misfolding: application to prion-related disorders and beyond. Trends Neurosci. 2002, 25 (8): 390-394.
Article
CAS
Google Scholar
Wille H, Michelitsch MD, Guenebaut V, Supattapone S, Serban A, Cohen FE, Agard DA, Prusiner SB: Structural studies of the scrapie prion protein by electron crystallography. Proc Natl Acad Sci USA. 2002, 99 (6): 3563-3568.
Article
PubMed Central
CAS
Google Scholar
Apetri AC, Maki K, Roder H, Surewicz WK: Early intermediate in human prion protein folding as evidenced by ultrarapid mixing experiments. J Am Chem Soc. 2006, 128 (35): 11673-11678.
Article
PubMed Central
CAS
Google Scholar
Apetri AC, Surewicz K, Surewicz WK: The effect of disease-associated mutations on the folding pathway of human prion protein. J Biol Chem. 2004, 279 (17): 18008-18014.
Article
CAS
Google Scholar
Gerber R, Tahiri-Alaoui A, Hore PJ, James W: Oligomerization of the human prion protein proceeds via a molten globule intermediate. J Biol Chem. 2007, 282 (9): 6300-6307.
Article
CAS
Google Scholar
Hosszu LL, Trevitt CR, Jones S, Batchelor M, Scott DJ, Jackson GS, Collinge J, Waltho JP, Clarke AR: Conformational properties of beta -PrP. J Biol Chem. 2009
Google Scholar
Jenkins DC, Sylvester ID, Pinheiro TJ: The elusive intermediate on the folding pathway of the prion protein. Febs J. 2008, 275 (6): 1323-1335.
Article
CAS
Google Scholar
Stohr J, Weinmann N, Wille H, Kaimann T, Nagel-Steger L, Birkmann E, Panza G, Prusiner SB, Eigen M, Riesner D: Mechanisms of prion protein assembly into amyloid. Proc Natl Acad Sci USA. 2008, 105 (7): 2409-2414.
Article
PubMed Central
Google Scholar
Campbell TA, Palmer MS, Will RG, Gibb WR, Luthert PJ, Collinge J: A prion disease with a novel 96-base pair insertional mutation in the prion protein gene. Neurology. 1996, 46 (3): 761-766.
Article
CAS
Google Scholar
Cochran EJ, Bennett DA, Cervenakova L, Kenney K, Bernard B, Foster NL, Benson DF, Goldfarb LG, Brown P: Familial Creutzfeldt-Jakob disease with a five-repeat octapeptide insert mutation. Neurology. 1996, 47 (3): 727-733.
Article
CAS
Google Scholar
Goldfarb LG, Brown P, Little BW, Cervenakova L, Kenney K, Gibbs CJ, Gajdusek DC: A new (two-repeat) octapeptide coding insert mutation in Creutzfeldt-Jakob disease. Neurology. 1993, 43 (11): 2392-2394.
Article
CAS
Google Scholar
Goldfarb LG, Brown P, McCombie WR, Goldgaber D, Swergold GD, Wills PR, Cervenakova L, Baron H, Gibbs CJ, Gajdusek DC: Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene. Proc Natl Acad Sci USA. 1991, 88 (23): 10926-10930.
Article
PubMed Central
CAS
Google Scholar
Laplanche JL, Delasnerie-Laupretre N, Brandel JP, Dussaucy M, Chatelain J, Launay JM: Two novel insertions in the prion protein gene in patients with late-onset dementia. Hum Mol Genet. 1995, 4 (6): 1109-1111.
Article
CAS
Google Scholar
Lawson VA, Priola SA, Meade-White K, Lawson M, Chesebro B: Flexible N-terminal region of prion protein influences conformation of protease-resistant prion protein isoforms associated with cross-species scrapie infection in vivo and in vitro. J Biol Chem. 2004, 279 (14): 13689-13695.
Article
CAS
Google Scholar
Hundt C, Peyrin JM, Haik S, Gauczynski S, Leucht C, Rieger R, Riley ML, Deslys JP, Dormont D, Lasmezas CI: Identification of interaction domains of the prion protein with its 37-kDa/67-kDa laminin receptor. Embo J. 2001, 20 (21): 5876-5886.
Article
PubMed Central
CAS
Google Scholar
Madhavaiah C, Verma S: Self-aggregation of reverse bis peptide conjugate derived from the unstructured region of the prion protein. Chem Commun (Camb). 2004, 638-639. 6
Bossers A: Prion Disease: Susceptibility and Transmissibility; In vivo and in vitro studies with sheep scrapie. 1999, PhD. University of Utrecht
Google Scholar
Bossers A, Belt P, Raymond GJ, Caughey B, de Vries R, Smits MA: Scrapie susceptibility-linked polymorphisms modulate the in vitro conversion of sheep prion protein to protease-resistant forms. Proc Natl Acad Sci USA. 1997, 94 (10): 4931-4936.
Article
PubMed Central
CAS
Google Scholar
Bossers A, Schreuder BE, Muileman IH, Belt PB, Smits MA: PrP genotype contributes to determining survival times of sheep with natural scrapie. J Gen Virol. 1996, 77 (Pt 10): 2669-2673.
Article
CAS
Google Scholar
Goldmann W, Hunter N, Smith G, Foster J, Hope J: PrP genotypes and the Sip gene in Cheviot sheep form the basis for scrapie strain typing in sheep. Ann N Y Acad Sci. 1994, 724: 296-299.
Article
CAS
Google Scholar
Hunter N, Foster JD, Goldmann W, Stear MJ, Hope J, Bostock C: Natural scrapie in a closed flock of Cheviot sheep occurs only in specific PrP genotypes. Arch Virol. 1996, 141 (5): 809-824.
Article
CAS
Google Scholar
Surewicz WK, Jones EM, Apetri AC: The emerging principles of Mammalian prion propagation and transmissibility barriers: insight from studies in vitro. Acc Chem Res. 2006, 39 (9): 654-662.
Article
CAS
Google Scholar
Wong C, Xiong LW, Horiuchi M, Raymond L, Wehrly K, Chesebro B, Caughey B: Sulfated glycans and elevated temperature stimulate PrP(Sc)-dependent cell-free formation of protease-resistant prion protein. Embo J. 2001, 20 (3): 377-386.
Article
PubMed Central
CAS
Google Scholar
Chabry J, Caughey B, Chesebro B: Specific inhibition of in vitro formation of protease-resistant prion protein by synthetic peptides. J Biol Chem. 1998, 273 (21): 13203-13207.
Article
CAS
Google Scholar
Chabry J, Priola SA, Wehrly K, Nishio J, Hope J, Chesebro B: Species-independent inhibition of abnormal prion protein (PrP) formation by a peptide containing a conserved PrP sequence. J Virol. 1999, 73 (8): 6245-6250.
PubMed Central
CAS
Google Scholar
Ziegler J, Viehrig C, Geimer S, Rosch P, Schwarzinger S: Putative aggregation initiation sites in prion protein. FEBS Lett. 2006, 580 (8): 2033-2040.
Article
CAS
Google Scholar
Satheeshkumar KS, Jayakumar R: Sonication induced sheet formation at the air-water interface. Chem Commun (Camb). 2002, 2244-2245. 19
Bosques CJ, Imperiali B: The interplay of glycosylation and disulfide formation influences fibrillization in a prion protein fragment. Proc Natl Acad Sci USA. 2003, 100 (13): 7593-7598.
Article
PubMed Central
CAS
Google Scholar
van Keulen LJ, Schreuder BE, Meloen RH, Poelen-van den Berg M, Mooij-Harkes G, Vromans ME, Langeveld JP: Immunohistochemical detection and localization of prion protein in brain tissue of sheep with natural scrapie. Vet Pathol. 1995, 32 (3): 299-308.
Article
CAS
Google Scholar
Goldmann W, Hunter N, Martin T, Dawson M, Hope J: Different forms of the bovine PrP gene have five or six copies of a short, G-C-rich element within the protein-coding exon. J Gen Virol. 1991, 72 (Pt 1): 201-204.
Article
CAS
Google Scholar
Rigter A, Priem J, Timmers-Parohi D, Langeveld JP, Bossers A: Mapping functional prion-prion protein interaction sites using prion protein based peptide-arrays. Methods Mol Biol. 2009, 570: 257-271.
Article
CAS
Google Scholar
Moudjou M, Frobert Y, Grassi J, La Bonnardiere C: Cellular prion protein status in sheep: tissue-specific biochemical signatures. J Gen Virol. 2001, 82 (Pt 8): 2017-2024.
Article
CAS
Google Scholar
Langeveld JP, Jacobs JG, Erkens JH, Bossers A, van Zijderveld FG, van Keulen LJ: Rapid and discriminatory diagnosis of scrapie and BSE in retro-pharyngeal lymph nodes of sheep. BMC Vet Res. 2006, 2: 19-
Article
PubMed Central
Google Scholar
Harmeyer S, Pfaff E, Groschup MH: Synthetic peptide vaccines yield monoclonal antibodies to cellular and pathological prion proteins of ruminants. J Gen Virol. 1998, 79 (Pt 4): 937-945.
Article
CAS
Google Scholar
Feraudet C, Morel N, Simon S, Volland H, Frobert Y, Creminon C, Vilette D, Lehmann S, Grassi J: Screening of 145 anti-PrP monoclonal antibodies for their capacity to inhibit PrPSc replication in infected cells. J Biol Chem. 2005, 280 (12): 11247-11258.
Article
CAS
Google Scholar