Scrapie is a deadly, degenerative and transmissible disease that affects the central nervous system of sheep and goats.

Scrapie has been known since the 18th century. This disease has been present in sheep and goats for centuries and has been observed worldwide, but especially in Western Europe and North America. In French the disease is called Tremblante and in German Traberkrankheit.

Severe itching

The name Scrapie is derived from one of the symptoms. Due to severe itching, sick animals scrape their skin against fences or other fixed objects. Other symptoms are a dry coat and skin, and severe weight loss. Movement disorders can also occur. However, these are not as pronounced as with BSE in cattle; usually only one animal of the herd is affected.

Long incubation time

Scrapie has an incubation period of more than two years. As a result, the disease symptoms are only seen in adult animals. In non-susceptible animals, the pathogen has virtually no chance of causing disease symptoms during their relatively short lives.

Prion disease

There are classical forms and atypical forms of scrapie. Scrapie, like BSE, is a transmissible spongiform encephalopathy (TSE) or prion disease. Prion diseases are very unusual; unlike bacterial, viral or parasitic infections, prion diseases are caused by a protein that is normally present in the host. This protein is known as a prion protein or simply PrP.

Spreading the infection

Scrapie is an infectious disease. The nature of the agent (the prion) that causes Scrapie is not fully understood, and the mode of transmission is not entirely clear. It has been established that the Scrapie agent can survive several years in the environment, that it is relatively resistant to disinfectants and that host genetic (hereditary) factors mainly determine the susceptibility to the disease. In sheep, the assumption is that an important route of horizontal and vertical transmission is via the placental material (afterbirth) of infected ewes during lambing.

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Other publications

Hicham Filali, Inmaculada Martin-Burriel, Frank Harders, Luis Varona, Carlos Hedman, Diego R Mediano, Marta Monzón, Alex Bossers, Juan J Badiola and Rosa Bolea. 2013. Gene expression profiling of mesenteric lymph nodes from naturally-infected scrapie sheep. BMC Genomics 15: 59. WOT-01-002-001.01

Bannach, O.; Birkmann, E.; Reinartz, E.; Karl-Erich, J.; Langeveld, J.P.M.; Rohwer, R.G.; Gregori, L.; Terry, L.A.; Willbold, D.; Riesner, D. (2012); Detection of Prion Protein Particles in Blood Plasma of Scrapie Infected Sheep; PLoS One 7 (5). - p. 8.Octrooinummer: e36620.

Tang, Y.; gielbert, A.; Jacobs, J.G.; Baron, T.; Andreoletti, O.; Langeveld, J.P.M.; Sauer, M.J. (2012); All major prion types recognised by a multiplex immunofluorometric assay for disease screening and confirmation in sheep; Journal of Immunological Methods 380 (1-2). - p. 30 - 39.

Bouzalas, I.; Lörtscher, F.; Dovas, C.; Oevermann, A.; Langeveld, J.P.M.; Papanastassopoulou, M.; Papadopoulos, O.; Zurbriggen, A.; Seuberlich, T. (2011); A distinct proteinase K resistant prion protein fragment in goats with no signs of disease in a classical scrapie outbreak; Journal of Clinical Microbiology 49 (6). - p. 2109 - 2115.

Filali, H.; Martin-Burriel, I.; Harders, F.; Varona, L.; Lyahyai, J.; Zaragoza, P.; Pumarola, M.; Badiola, J.J.; Bossers, A.; Bolea, R. (2011); Gene expression profiling en association with prion-related lesions in the medulla oblongata of symptomatic natural scrapie animals.; PLoS One 6 (5). - p. 12.Octrooinummer: e19909.

Goldmann, W.; Ryan, K.; Stewart, P.; Parnham, D.; Xicohtencatl, R.; Fernandez, N.; Saunders, G.; Windl, O.; Gonzalez, L.; Bossers, A. (2011); Caprine prion genen polymorphisms are associated with decreased incidence of classical scrapie in goat herds in the United Kingdom.; Veterinary Research 42 . - p. 8pp.

Jacobs, J.G.; Bossers, A.; Rezaei, H.; Van Keulen, L.; McCutcheon, S.; Sklaviadis, T.; Lantier, I.; Berthon, P.; Lantier, F.; Zijderveld, F.G. van; Langeveld, J.P.M. (2011); Proteinase K resistant material in ARR/VRQ sheep brain affected with classical scrapie is composed mainly of VRQ prion protein; Journal of Virology 85 . - p. 12537 - 12546.

Melchior, M.B.; Hagenaars, T.J.; Davidse, A.; Keulen, L. van; Bossers, A.; Zijderveld, F.G. van (2011); Active surveillance for scrapie in the Netherlands: effect of a breeding programme on the prevalence of scrapie in sheep (2002-2010); Tijdschrift voor Diergeneeskunde 136 (2). - p. 84 - 93.

Nodelijk, G.; Roermund, H.J.W. van; Keulen, L.J.M. van; Engel, B.; Vellema, P. (2011); Breeding with resistant rams leads to rapid control of classical scrapie in affected sheep flocks; Veterinary Research 42 (1). - p. 5.

Rigter, A.; Priem, J.; Langeveld, J.P.M.; Bossers, A. (2011); Prion protein self-interaction in prion disease therapy approaches; Veterinary Quarterly 31 (3). - p. 115 - 128.

Gubbins, S.; Touzeau, S.; Hagenaars, T.J. (2010); the role of mathematical modelling in understanding the epidemiology and control of sheep transmissible spongiform encephalopathies: a review; Veterinary Research 41 (4). - p. 41:42.

Hagenaars, T.J.; Melchior, M.B.; Bossers, A.; Davidse, A.; Engel, B.; Zijderveld, F.G. van (2010); Scrapie prevalence in sheep of susceptible genotype is declining in a population subject to breeding for resistance; BMC Veterinary Research 6 . - p. 25.

Melchior, M.B.; Windig, J.J.; Hagenaars, T.J.; Bossers, A.; Davidse, A.; Zijderveld, F.G. van (2010); Eradication of scrapie with selective breeding: are we nearly there?; BMC Veterinary Research 6 . - p. 24.

Pirisinu, L.; Di Bari, M.; Marcon, S.; Vaccari, G.; D'Agostino, C.; Fazzi, P.; Esposito, E.; Cardone, F.; Langeveld, J.P.M.; Agrimi, U.; Nonno, R. (2010); A new method for the Characterization of Strain-Specific Conformational Stability of Protease-Sensitive and Protease Resistant PrPSc; PLoS One 5 . - p. 12723.

Polak, M.P.; Larska, M.; Langeveld, J.P.M.; Buschmann, A.; Groschup, M.H.; Zmudzinski, J.F. (2010); Diagnosis of the first cases of scrapie in Poland; The Veterinary Journal 186 (1). - p. 47 - 52.

Rigter, A.; Langeveld, J.P.M.; Zijderveld, F.G. van; Bossers, A. (2010); Prion Protein Self Interactions; a gateway to novel therapeutic strategies?; Vaccine 28 (49). - p. 7810 - 7823.

Tang, Y.; Thorne, J.; Whatling, K.L.; Jacobs, J.G.; Langeveld, J.P.M.; Sauer, M. (2010);A single step multiplex immunofluorometric assay for differential diagnosis of BSE and scrapie; Journal of Immunological Methods 356 (1-2). - p. 29 - 38.

Cali, I.; Castellani, R.; Alshekhlee, A.; Cohen, Y.; Blevins, J.; Yuan, J.; Langeveld, J.P.M.; Parchi, P.; Safar, J.G.; Zou, W.Q.; Gambetti, P. (2009); Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics; Brain 132 . - p. 2643 - 2658.

Orrú, C.D.; Wilham, J.M.; Hughson, A.G.; Raymond, L.D.; McNally, K.L.; Bossers, A.; Ligios, C.; Caughey, B. (2009); Human Variant Creutzfeldt-Jakob disease and sheep scrapie PrP (res) detection using seeded conversion of recombinant prion protein.; Protein Engineering, Design & Selection 22 (8). - p. 515 - 521.

Rigter, A.; Priem, J.; Timmers-Parohi, D.; Langeveld, J.P.M.; Zijderveld, F.G. van; Bossers, A. (2009); Prion protein self-peptides modulate prion interactions and conversion; BMC Biochemistry 10 . - p. 29.

Vaccari, G.; Panagiotidis, C.H.; Acin, C.; Peletto, S.; Barillet, F.; Acutis, P.; Bossers, A.; Langeveld, J.P.M.; Keulen, L.J.M. van; Sklaviadis, T.; Badiola, J.J.; Andreoletti, O.; Groschup, M.H.; Agrimi, U.; Foster, J.; Goldmann, W. (2009); State-of-the-art review of goat TSE in the European Union, with special emphasis on PRNP genetics and epidemiology; Veterinary Research 40 (5). - p. 48.

Acutis, P.L.; Colussi, S.; Santagada, G.; Laurenza, C.; Maniaci, M.G.; Riina, M.V.; Peletto, S.; Goldmann, W.; Bossers, A.; Caramelli, M.; Cristoferi, I.; Maione, S.; Sacchi, P.; Rasero, R. (2008); Genetic variability of the PRNP gene in goat breeds from Northern and Southern Italy; Journal of Applied Microbiology 104 (6). - p. 1782 - 1789.

Keulen, L.J.M. van; Bossers, A.; Zijderveld, F.G. van (2008); TSE pathogenesis in cattle and sheep; Veterinary Research 39 (4). - p. 24.

Keulen, L.J.M. van; Vromans, M.E.W.; Dolstra, C.H.; Bossers, A.; Zijderveld, F.G. van (2008); Pathogenesis of bovine spongiform encephalopathy in sheep; Archives of Virology 153 (3). - p. 445 - 453.

Espinosa, J.C.; Andreoletti, O.; Castilla, J.; Herva, M.E.; Morales, M.; Alamillo, E.; San-Segundo, F.D.; Lacroux, C.; Lugan, S.; Salguero, F.J.; Langeveld, J.P.M.; Torres, J.M. (2007); Sheep-passaged bovine spongiform encephalopathy agent exhibits altered pathobiological properties in bovine-PrP transgenic mice; Journal of Virology 81 (2). - p. 835 - 843.

Nodelijk, G.; Hagenaars, T.J.; Keulen, L.J.M. van; Roermund, H.J.W. van; Jong, M.C. de (2007); Evaluation of the effect of the Dutch national scrapie control programme; UK : Chapter 3 of EU report ScrapieFreeSheep,

Rigter, A.; Langeveld, J.P.M.; Timmers-Parohi, D.; Jacobs, J.G.; Moonen, P.L.J.M.; Bossers, A. (2007); Mapping of possible prion protein self interaction domains using peptide arrays; Biochemistry 8 . - p. 6.

Acutis, P.L.; Bossers, A.; Priem, J.; Riina, M.V.; Peletto, S.; Mazza, M.; Casalone, C.; Forloni, G.; Ru, G.; Caramelli, M. (2006); Identification of prion protein gene polymorphisms in goats from Italian scrapie outbreaks; Journal of General Virology 87 . - p. 1029 - 1033.

Gretzschel, A.; Buschmann, A.; Langeveld, J.P.M.; Groschup, M.H. (2006); Immunological characterization of abnormal prion protein from atypical scrapie cases in sheep using a panel of monoclonal antibodies; Journal of General Virology 87 . - p. 3715 - 3722.

Hagenaars, T.J.; Donnelly, C.; Ferguson, N.M. (2006) Epidemiological analysis of data for scrapie in Great Britain, Epidemiology and Infection 134 (2). - p. 359 - 367.

Jeffrey, M.; Martin, S.; Gonzalez, L.; Foster, J.; Langeveld, J.P.M.; Zijderveld, F.G. van; Grassi, J.; Hunter, N. (2006); Immunohistochemical features of Prp(d) accumulation in natural and experimental goat transmissible spongiform encephalopathies; Journal of Comparative Pathology 134 (2-3). - p. 171 - 181.

Langeveld, J.P.M.; Jacobs, J.G.; Erkens, J.H.F.; Bossers, A.; Zijderveld, F.G. van; Keulen, L.J.M. van (2006); Rapid and discriminatory diagnosis of scrapie and BSE in retro-pharyngeal lymph nodes of sheep; BMC Veterinary Research 2 . - p. 19.

Raymond, G.J.; Olsen, E.A.; Lee, K.S.; Raymond, L.D.; Bryant, P.K.; Baron, G.S.; Caughey, W.S.; Kocisko, D.A.; McHolland, L.E.; Favara, C.; Langeveld, J.P.M.; Zijderveld, F.G. van; Mayer, R.T.; Miller, M.W.; Williams, E.S.; Caughey, B. (2006); Inhibition of protease-resistant prion protein formation in a transformed deer cell line infected with chronic wasting disease; Journal of Virology 80 (2). - p. 596 - 604.

Rigter, A.; Bossers, A. (2005); Sheep scrapie susceptibility-linked polymorphisms do not modulate the initial binding of cellular to disease-associated prion protein prior to conversion; Journal of General Virology 86 (9). - p. 2627 - 2634.

Sharpe, A.; McElroy, M.; Langeveld, J.P.M.; Bassett, H.; O'Donoghue, A.M.; Sweeney, T. (2005); Immunohistochemical studies of scrapie archival material from Irish ARQ/ARQ sheep for evidence of bovine spongiform encephalopathy-derived disease; Research in Veterinary Science 79 (1). - p. 29 - 35.

Thuring, C.M.A.; Keulen, L.J.M. van; Langeveld, J.P.M.; Vromans, M.E.W.; Zijderveld, F.G. van; Sweeney, T. (2005); Immunohistochemical distinction between preclinical bovine spongiform encephalopathy and scrapie infection in sheep; Journal of Comparative Pathology 132 (1). - p. 59- - 69.

Veerhuis, R.; Boshuizen, R.S.; Morbin, M.; Mazzoleni, G.; Hoozemans, J.J.; Langedijk, J.P.; Tagliavini, F.; Langeveld, J.P.M.; Eikelenboom, P. (2005); Activation of human microglia by fibrillar prion protein-related peptides is enhanced by amyloid-associated factors SAP and C1q; Neurobiology of Disease 19 (1-2). - p. 273 - 282.

Acutis, P.L.; Sbaiz, L.; Verburg, F.J.; Riina, M.V.; Ru, G.; Moda, G.; Caramelli, M.; Bossers, A. (2004); Low frequency of the scrapile resistance-associated allele and presence of lysine-171 allele of the prion protein gene in Italian Biellese ovine breed; Journal of General Virology 85 . - p. 3165 - 3172.

Bate, C.; Langeveld, J.P.M.; Williams, A. (2004); Manipulation of PrPres production in scrapie-infected neuroblastoma cells; Journal of Neuroscience Methods 138 (1-2). - p. 217 - 223.

Thuring, C.M.A.; Erkens, J.H.F.; Jacobs, J.G.; Bossers, A.; Keulen, L.J.M. van; Garssen, G.J.; Zijderveld, F.G. van; Ryder, S.J.; Groschup, M.H.; Sweeney, T.; Langeveld, J.P.M. (2004); Discrimination between scrapie and bovine spongiform encephalopathy in sheep by molecular size, immunoreactivity, and glycoprofile of prion protein; Journal of Clinical Microbiology 42 (3). - p. 972 - 980.

Hagenaars, T.J.; Donelly, C.A.; Ferguson, N.M.; Anderson, R.M. (2003); Dynamics of a scrapie outbreak in a flock of Romanov sheep-estimation of transmission parameters; Epidemiology and Infection 131 . - p. 1015 - 1022.

Langeveld, J.P.M.; Wang, J.J.; Wiel, D.F.M. van de; Shih, G.C.; Garssen, G.J.; Bossers, A.; Shih, J.C.H. (2003); Enzymatic degradation of prion protein in brain stem from infected cattle and sheep; The Journal of Infectious Diseases 188 (11). - p. 1782 - 1789.

Bate, C.; Boshuizen, R.S.; Langeveld, J.P.M.; Williams, A. (2002); Temporal and spatial relationship between the death of PrP-damaged neurones and microglial activation; NeuroReport 13 (13). - p. 1695 - 1700.

Keulen, L.J.M. van; Vromans, M.E.W.; Zijderveld, F.G. van (2002); Early and late pathogenesis of natural scrapie infection in sheep; Apmis 110 (1). - p. 23 - 32.

Andreoletti, O.; Berthon, P.; Marc, D.; Sarradin, P.; Grosclaude, J.; Keulen, L. van; Schelcher, F.; Elsen, J.M.; Lantier, F. (2000); Early accumulation of PrPSc in gut-associated lymphoid and nervous tissues of susceptible sheep from a Romanov flock with natural scrapie; Journal of general virology 81 Part 12, 3115-3126

Garssen, G.J.; Keulen, L.J.M. van; Farquhar, C.F.; Smits, M.A.; Jacobs, J.G.; Bossers, A.; Meloen, R.H.; Langeveld, J.P.M. (2000); Applicability of three anti-PrP peptide sera including staining of tonsils and brainstem of sheep with scrapie; Microscopy research and technique 50, 2000, 32-39

Keulen, L.J. van; Schreuder, B.E.; Vromans, M.E.; Langeveld, J.P.; Smits, M.A. (2000); Pathogenesis of natural scrapie in sheep; Archives of Virology, Supplement, 57-71

Bossers, A. (1999); Prion diseases: Susceptibility and transmissibility. In vivo and in vitro studies with sheep scrapie; Thesis, 1999. - ISBN 90-393-2108-6

Bossers, A.; Harders, F.L.; Smits, M.A. (1999); PrP genotype frequencies of the most dominant sheep breed in a country free from scrapie; Archives of virology 144, 1999, 829-834

Keulen, L.J.M. van; Schreuder, B.E.C.; Vromans, M.E.W.; Langeveld, J.P.M.; Smits, M.A. (1999); Scrapie-associated prion protein in the gastro-intestinal tract of sheep with natural scrapie; Journal comparative pathology 121, 1999, 55-63

Roels, S.; Vanopdenbosch, E.; Langeveld, J.P.M.; Schreuder, B.E.C. (1999); Immunohistochemical evaluation of tonsillar tissue for preclinical screening of scrapie based on surveillance in Belgium; Veterinary Record 145 (18). - p. 524 - 525.

Schreuder, B.E.C.; Geertsma, R.E.; Keulen, L.J.M. van; Asten, J.A.A.M. van; Enthoven, P.; Oberthür, R.C.; Koeijer, A.A. de; Osterhaus, A.D.M.E. (1998); Studies on the efficacy of hyperbaric rendering procedures in inactivating bovine spongiform encephalopathy (BSE) and scrapie agents; Veterinary Record 142 (19). - p. 474 - 480.

Schreuder, B.E.C.; Keulen, L.J.M. van; Vromans, M.E.W.; Langeveld, J.P.M.; Smits, M.A. (1998); Tonsillar biopsy and PrPSc detection in the preclinical diagnosis of scrapie; Veterinary Record 142 (21). - p. 564 - 568.

Raymond, G.J.; Hope, J.; Kocisko, D.A.; Priola, S.A.; Raymond, L.D.; Bossers, A.; Ironside, J.; Will, R.G.; Chen, S.G.; Petersen, R.B.; Gambetti, P.; Rubenstei, R.; Smits, M.A.; Lansbury, P.T.; Caughey, B. (1997); Molecular assessment of the potential transmissibilities of BSE and scrapie to humans; Nature 388, 1997, 285-288

Smits, M.A.; Bossers, A.; Schreuder, B.E.C. (1997); Prion protein and scrapie susceptibility; Veterinary quarterly 19, 1997, 101-105

Keulen, L.J.M. van; Schreuder, B.E.C.; Meloen, R.H.; Mooij-Harkes, G.; Vromans, M.E.W.; Langeveld, J.P.M. (1996); Immunohistochemical detection of prion protein in lymphoid tissues of sheep with natural scrapie; Journal clinical microbiology 34, 1996, 1228-1231

Belt, P.B.G.M.; Muileman, I.H.; Schreuder, B.E.C.; Bos-de Ruijter, J.; Gielkens, A.L.J.; Smits, M.A. (1995); Identification of five allelic variants of the sheep PrP gene and their association with natural scrapie; Journal of general virology 76, 1995, 509-517

Keulen, L.J.M.; Schreuder, B.E.C.; Meloen, R.H.; Poelen-van der Berg, M.; Mooij-Harkes, G.; Vromans, M.E.W.; Langeveld, J.P.M. (1995); lmmunohistochemical detection and localization of prion protein in brain tissue of sheep with natural scrapie; Veterinary pathology 32, 1995, 299-308