Breeding programmes to promote resistance to classical scrapie, similar to those for sheep in existingtransmissible spongiform encephalopathies (TSE) regulations, have not been established in goats. TheEuropean Commission requested a scientific opinion from EFSA on the current knowledge of geneticresistance to TSE in goats. An evaluation tool, which considers both the weight of evidence andstrength of resistance to classical scrapie of alleles in the goatPRNPgene, was developed and appliedto nine selected alleles of interest. Using the tool, the quality and certainty of thefield andexperimental data are considered robust enough to conclude that the K222, D146 and S146 allelesboth confer genetic resistance against classical scrapie strains known to occur naturally in the EU goatpopulation, with which they have been challenged both experimentally and underfield conditions. Theweight of evidence for K222 is greater than that currently available for the D146 and S146 alleles andfor the ARR allele in sheep in 2001. Breeding for resistance can be an effective tool for controllingclassical scrapie in goats and it could be an option available to member states, both at herd andpopulation levels. There is insufficient evidence to assess the impact of K222, D146 and S146 alleleson susceptibility to atypical scrapie and bovine spongiform encephalopathy (BSE), or on health andproduction traits. These alleles are heterogeneously distributed across the EU Member States and goatbreeds, but often at low frequencies (<10%). Given these low frequencies, high selection pressuremay have an adverse effect on genetic diversity so any breeding for resistance programmes should bedeveloped at Member States, rather than EU level and their impact monitored, with particular attentionto the potential for any negative impact in rare or small population breeds
Acín Cristina, Martín-Burriel Inmaculada, Monleón Eva, Lyahyai Jaber, Pitarch José Luis, Serrano Carmen, Monzón Marta, Zaragoza Pilar, Badiola Juan José, Prion Protein Gene Variability in Spanish Goats. Inference through Susceptibility to Classical Scrapie Strains and Pathogenic Distribution of Peripheral PrPsc, 10.1371/journal.pone.0061118
Acutis P. L., Identification of prion protein gene polymorphisms in goats from Italian scrapie outbreaks, 10.1099/vir.0.81440-0
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., Genetic variability of the PRNP gene in goat breeds from Northern and Southern Italy, 10.1111/j.1365-2672.2007.03703.x
Acutis Pier Luigi, Martucci Francesca, D'Angelo Antonio, Peletto Simone, Colussi Silvia, Maurella Cristiana, Porcario Chiara, Iulini Barbara, Mazza Maria, Dell'Atti Luana, Zuccon Fabio, Corona Cristiano, Martinelli Nicola, Casalone Cristina, Caramelli Maria, Lombardi Guerino, Resistance to classical scrapie in experimentally challenged goats carrying mutation K222 of the prion protein gene, 10.1186/1297-9716-43-8
Agrimi Umberto, Ru Giuseppe, Cardone Franco, Pocchiari Maurizio, Caramelli Maria, Epidemic of transmissible spongiform encephalopathy in sheep and goats in Italy, 10.1016/s0140-6736(98)04545-0
Aguilar-Calvo P., Espinosa J. C., Pintado B., Gutierrez-Adan A., Alamillo E., Miranda A., Prieto I., Bossers A., Andreoletti O., Torres J. M., Caughey B. W., Role of the Goat K222-PrPC Polymorphic Variant in Prion Infection Resistance, 10.1128/jvi.02074-13
Aguilar-Calvo Patricia, Fast Christine, Tauscher Kerstin, Espinosa Juan-Carlos, Groschup Martin H., Nadeem Muhammad, Goldmann Wilfred, Langeveld Jan, Bossers Alex, Andreoletti Olivier, Torres Juan-María, Effect of Q211and K222PRNPPolymorphic Variants in the Susceptibility of Goats to Oral Infection With Goat Bovine Spongiform Encephalopathy, 10.1093/infdis/jiv112
Aguilar-Calvo Patricia, Espinosa Juan-Carlos, Andréoletti Olivier, González Lorenzo, Orge Leonor, Juste Ramón, Torres Juan-María, Goat K222-PrPC polymorphic variant does not provide resistance to atypical scrapie in transgenic mice, 10.1186/s13567-016-0380-7
Arsac Jean-Noël, Andreoletti Olivier, Bilheude Jean-Marc, Lacroux Caroline, Benestad Sylvie L., Baron Thierry, Similar Biochemical Signatures and Prion Protein Genotypes in Atypical Scrapie and Nor98 Cases, France and Norway, 10.3201/eid1301.060393
Arsac Jean-Noël, Bétemps Dominique, Morignat Eric, Féraudet Cécile, Bencsik Anna, Aubert Denise, Grassi Jacques, Baron Thierry, Transmissibility of Atypical Scrapie in Ovine Transgenic Mice: Major Effects of Host Prion Protein Expression and Donor Prion Genotype, 10.1371/journal.pone.0007300
Asante Emmanuel A., Smidak Michelle, Grimshaw Andrew, Houghton Richard, Tomlinson Andrew, Jeelani Asif, Jakubcova Tatiana, Hamdan Shyma, Richard-Londt Angela, Linehan Jacqueline M., Brandner Sebastian, Alpers Michael, Whitfield Jerome, Mead Simon, Wadsworth Jonathan D. F., Collinge John, A naturally occurring variant of the human prion protein completely prevents prion disease, 10.1038/nature14510
Asante E. A., BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein, 10.1093/emboj/cdf653
Barillet F., Mariat D., Amigues Y., Faugeras R., Caillat H., Moazami-Goudarzi K., Rupp R., Babilliot J. M., Lacroux C., Lugan S., Schelcher F., Chartier C., Corbiere F., Andreoletti O., Perrin-Chauvineau C., Identification of seven haplotypes of the caprine PrP gene at codons 127, 142, 154, 211, 222 and 240 in French Alpine and Saanen breeds and their association with classical scrapie, 10.1099/vir.0.006114-0
Baylis M., Ross A., Chong A., Goldmann W., Hunter N., Smith A., McLean A. R., Cairns D., Houston F., Scrapie epidemic in a fully PrP-genotyped sheep flock, 10.1099/0022-1317-83-11-2907
Baylis M., Risk of scrapie in British sheep of different prion protein genotype, 10.1099/vir.0.79876-0
Beck J A, Campbell T A, Adamson G, Poulter M, Uphill J B, Molou E, Collinge J, Mead S, Association of a null allele of SPRN with variant Creutzfeldt-Jakob disease, 10.1136/jmg.2008.061804
Behrens A., Absence of the prion protein homologue Doppel causes male sterility, 10.1093/emboj/cdf386
Bellworthy S. J., Hawkins S. A. C., Green R. B., Blamire I., Dexter G., Dexter I., Lockey R., Ryder S., Berthelin-Baker C., Simmons M. M., Jeffrey M., Tissue distribution of bovine spongiform encephalopathy infectivity in Romney sheep up to the onset of clinical disease after oral challenge, 10.1136/vr.156.7.197
Belt P. B. G. M., Muileman I. H., Schreuder B. E. C., Bos-de Ruijter J., Gielkens A. L. J., Smits M. A., Identification of five allelic variants of the sheep PrP gene and their association with natural scrapie, 10.1099/0022-1317-76-3-509
Benestad Sylvie L, Austbø Lars, Tranulis Michael A, Espenes Arild, Olsaker Ingrid, Healthy goats naturally devoid of prion protein, 10.1186/1297-9716-43-87
Béringue Vincent, Vilotte Jean-Luc, Laude Hubert, Prion agent diversity and species barrier, 10.1051/vetres:2008024
Bessen, Journal of Virology, 66, 2096 (1992)
Biacabe Anne-Gaëlle, Jacobs Jorg G., Bencsik Anna, Langeveld Jan P.M., Baron Thierry G.M., H-Type Bovine Spongiform Encephalopathy : Complex Molecular Features and Similarities with Human Prion Diseases, 10.4161/pri.1.1.3828
Biljan Ivana, Giachin Gabriele, Ilc Gregor, Zhukov Igor, Plavec Janez, Legname Giuseppe, Structural basis for the protective effect of the human prion protein carrying the dominant-negative E219K polymorphism, 10.1042/bj20111940
Bishop MT, Hart P, Aitchison L, Baybutt HN, Plinston C, Thomson V, Tuzi NL, Head MW, Ironside JW, Will RG, Manson JC, Predicting susceptibility and incubation time of human-to-human transmission of vCJD, 10.1016/s1474-4422(06)70413-6
Bossers A., Schreuder B. E. C., Muileman I. H., Belt P. B. G. M., Smits M. A., PrP genotype contributes to determining survival times of sheep with natural scrapie, 10.1099/0022-1317-77-10-2669
Bossers A., Belt P. B. G. M., Raymond G. J., Caughey B., de Vries R., Smits M. A., Scrapie susceptibility-linked polymorphisms modulate the in vitro conversion of sheep prion protein to protease-resistant forms, 10.1073/pnas.94.10.4931
Bossers A., de Vries R., Smits M. A., Susceptibility of Sheep for Scrapie as Assessed by In Vitro Conversion of Nine Naturally Occurring Variants of PrP, 10.1128/jvi.74.3.1407-1414.2000
Boulton K., Moore R.C., Bishop S.C., Associations of PrP genotype with lamb production traits in four commercial breeds of British upland and crossing sheep, 10.1016/j.livsci.2009.09.007
Bouzalas I. G., Dovas C. I., Banos G., Papanastasopoulou M., Kritas S., Oevermann A., Papakostaki D., Evangelia C., Papadopoulos O., Seuberlich T., Koptopoulos G., Caprine PRNP polymorphisms at codons 171, 211, 222 and 240 in a Greek herd and their association with classical scrapie, 10.1099/vir.0.017350-0
Bruce M. E., Dickinson A. G., Biological Evidence that Scrapie Agent Has an Independent Genome, 10.1099/0022-1317-68-1-79
Bucalossi C., Cosseddu G., D'Agostino C., Di Bari M. A., Chiappini B., Conte M., Rosone F., De Grossi L., Scavia G., Agrimi U., Nonno R., Vaccari G., Assessment of the Genetic Susceptibility of Sheep to Scrapie by Protein Misfolding Cyclic Amplification and Comparison with Experimental Scrapie Transmission Studies, 10.1128/jvi.00241-11
Bueler, Molecular Medicine, 1, 19 (1994)
Canon J., Garcia D., Garcia-Atance M. A., Obexer-Ruff G., Lenstra J. A., Ajmone-Marsan P., Dunner S., , Geographical partitioning of goat diversity in Europe and the Middle East, 10.1111/j.1365-2052.2006.01461.x
Gutiérrez Adán A., Brun A., Pintado B., Ramírez M. A., Parra B., Doyle D., Rogers M., Salguero F. J., Castilla J., Sánchez C., Sánchez-Vizcaíno J. M., Torres J. M., Early detection of PrP res in BSE-infected bovine PrP transgenic mice, 10.1007/s00705-002-0958-4
Castilla Joaquín, Gonzalez-Romero Dennisse, Saá Paula, Morales Rodrigo, De Castro Jorge, Soto Claudio, Crossing the Species Barrier by PrPSc Replication In Vitro Generates Unique Infectious Prions, 10.1016/j.cell.2008.07.030
Chelle, Bulletin de l'Académie Vétérinaire de France, 15, 294 (1942)
Clouscard C., Beaudry P., Elsen J. M., Milan D., Dussaucy M., Bounneau C., Schelcher F., Chatelain J., Launay J. M., Laplanche J. L., Different allelic effects of the codons 136 and 171 of the prion protein gene in sheep with natural scrapie, 10.1099/0022-1317-76-8-2097
Colussi S., Vaccari G., Maurella C., Bona C., Lorenzetti R., Troiano P., Casalinuovo F., Di Sarno A., Maniaci M. G., Zuccon F., Nonno R., Casalone C., Mazza M., Ru G., Caramelli M., Agrimi U., Acutis P. L., Histidine at codon 154 of the prion protein gene is a risk factor for Nor98 scrapie in goats, 10.1099/vir.0.2008/004150-0
Corbiere F., Perrin-Chauvineau C., Lacroux C., Costes P., Thomas M., Bremaud I., Martin S., Lugan S., Chartier C., Schelcher F., Barillet F., Andreoletti O., PrP-associated resistance to scrapie in five highly infected goat herds, 10.1099/vir.0.047225-0
Dassanayake Rohana P., White Stephen N., Madsen-Bouterse Sally A., Schneider David A., O'Rourke Katherine I., Role of thePRNPS127 allele in experimental infection of goats with classical caprine scrapie, 10.1111/age.12291
de Rancourt M., Fois N., Lavín M.P., Tchakérian E., Vallerand F., Mediterranean sheep and goats production: An uncertain future, 10.1016/j.smallrumres.2005.08.012
de Vries F., Hamann N., Drogemuller C., Distl O., Borchers N., Reinecke S., Lupping W., Associations between the prion protein genotype and performance traits of meat breeds of sheep, 10.1136/vr.155.5.140
Detwiler, Revue scientifique et technique (International Office of Epizootics), 22, 121 (2003)
Dexter Glenda, Tongue Sue C, Heasman Lindsay, Bellworthy Susan J, Davis Andrew, Moore S Jo, Simmons Marion M, Sayers A Robin, Simmons Hugh A, Matthews Danny, The evaluation of exposure risks for natural transmission of scrapie within an infected flock, 10.1186/1746-6148-5-38
Di Bari M. A., Chianini F., Vaccari G., Esposito E., Conte M., Eaton S. L., Hamilton S., Finlayson J., Steele P. J., Dagleish M. P., Reid H. W., Bruce M., Jeffrey M., Agrimi U., Nonno R., The bank vole (Myodes glareolus) as a sensitive bioassay for sheep scrapie, 10.1099/vir.0.2008/005520-0
Dustan B. H., Spencer Y. I., Casalone C., Brownlie J., Simmons M. M., A Histopathologic and Immunohistochemical Review of Archived UK Caprine Scrapie Cases, 10.1354/vp.45-4-443
, Opinion of the Scientific Panel on biological hazards (BIOHAZ) on the Breeding programme for TSE resistance in sheep : Opinion of the Scientific Panel on biological hazards (BIOHAZ) on the Breeding programme for TSE resistance in sheep, 10.2903/j.efsa.2006.382
, Evaluation of the FoodEx, the food classification system applied to the development of the EFSA Comprehensive European Food Consumption Database : Evaluation of the FoodEx food classification system, 10.2903/j.efsa.2011.1970
, Use of the EFSA Comprehensive European Food Consumption Database in Exposure Assessment : Use of the Comprehensive Database, 10.2903/j.efsa.2011.2097
, Scientific and technical assistance on the provisional results of the study on genetic resistance to Classical scrapie in goats in Cyprus : Genetic resistance to Classical scrapie in goats in Cyprus, 10.2903/j.efsa.2012.2972
, Scientific Opinion on the scrapie situation in the EU after 10 years of monitoring and control in sheep and goats : Scrapie situation in the EU, 10.2903/j.efsa.2014.3781
, Scientific Opinion on a request for a review of a scientific publication concerning the zoonotic potential of ovine scrapie prions : Zoonotic potential scrapie, 10.2903/j.efsa.2015.4197
Eiden Martin, Soto Elizabeth, Mettenleiter Thomas C, Groschup Martin H, Effects of polymorphisms in ovine and caprine prion protein alleles on cell-free conversion, 10.1186/1297-9716-42-30
Eloit M., Adjou K., Coulpier M., Fontaine J. J., Hamel R., Lilin T., Messiaen S., Andreoletti O., Baron T., Bencsik A., Gaelle Biacabe A., Beringue V., Laude H., Le Dur A., Vilotte J. L., Comoy E., Deslys J. P., Grassi J., Simon S., Lantier F., Sarradin P., BSE agent signatures in a goat, 10.1136/vr.156.16.523-b
Elsen J.-M., Amigues Y., Schelcher F., Ducrocq V., Andreoletti O., Eychenne F., Tien Khang J. V., Poivey J.-P., Lantier F., Laplanche J.-L., Genetic susceptibility and transmission factors in scrapie: detailed analysis of an epidemic in a closed flock of Romanov, 10.1007/s007050050516
FAO (Food and Agriculture Organization), 2012. Phenotypic characterization of animal genetic resources. FAO Animal Production and Health Guidelines No. 11. Rome.
Fast C, Goldmann W, Berthon P, Tauscher K, Niedermeyer S, Andréoletti O, Lantier I, Rossignol C, Bossers A, Jacobs JG, Hunter N, Groschup MH, Lantier F and Langeveld JPM, in press. Protecting effect of PrP codons M142 and K222 in goats orally challenged with bovine spongiform encephalopathy prions.
Fediaevsky Alexandre, Tongue Sue C, Nöremark Maria, Calavas Didier, Ru Giuseppe, Hopp Petter, A descriptive study of the prevalence of atypical and classical scrapie in sheep in 20 European countries, 10.1186/1746-6148-4-19
Foster J., Dickinson A., The unusual properties of CH1641, a sheep-passaged isolate of scrapie, 10.1136/vr.123.1.5
Foster J., Hope J., Fraser H., Transmission of bovine spongiform encephalopathy to sheep and goats, 10.1136/vr.133.14.339
Foster J. D., Bruce M., McConnell I., Chree A., Fraser H., Detection of BSE infectivity in brain and spleen of experimentally infected sheep, 10.1136/vr.138.22.546
Foster J. D., Parnham D., Chong A., Goldmann W., Hunter N., Clinical signs, histopathology and genetics of experimental transmission of BSE and natural scrapie to sheep and goats, 10.1136/vr.148.6.165
Foster J. D., Parnham D. W., Hunter N., Bruce M., Distribution of the prion protein in sheep terminally affected with BSE following experimental oral transmission, 10.1099/0022-1317-82-10-2319
Fragkiadaki Eirini G, Vaccari Gabriele, Ekateriniadou Loukia V, Agrimi Umberto, Giadinis Nektarios D, Chiappini Barbara, Esposito Elena, Conte Michela, Nonno Romolo, PRNP genetic variability and molecular typing of natural goat scrapie isolates in a high number of infected flocks, 10.1186/1297-9716-42-104
GEORGIADOU S., ORTIZ-PELAEZ A., SIMMONS M. M., WINDL O., DAWSON M., NEOCLEOUS P., PAPASAVVA-STYLIANOU P., Goats with aspartic acid or serine at codon 146 of the PRNP gene remain scrapie-negative after lifetime exposure in affected herds in Cyprus, 10.1017/s0950268816002272
Goldmann W., Martin T., Foster J., Hughes S., Smith G., Hughes K., Dawson M., Hunter N., Novel polymorphisms in the caprine PrP gene: a codon 142 mutation associated with scrapie incubation period, 10.1099/0022-1317-77-11-2885
Goldmann Wilfred, Ryan Kelly, Stewart Paula, Parnham David, Xicohtencatl Rosa, Fernandez Nora, Saunders Ginny, Windl Otto, González Lorenzo, Bossers Alex, Foster James, Caprine prion gene polymorphisms are associated with decreased incidence of classical scrapie in goat herds in the United Kingdom, 10.1186/1297-9716-42-110
Goldmann W., Marier E., Stewart P., Konold T., Street S., Langeveld J., Windl O., Ortiz-Pelaez A., Prion protein genotype survey confirms low frequency of scrapie-resistant K222 allele in British goat herds, 10.1136/vr.103521
González Lorenzo, Martin Stuart, Sisó Sílvia, Konold Timm, Ortiz-Peláez Angel, Phelan Laura, Goldmann Wilfred, Stewart Paula, Saunders Ginny, Windl Otto, Jeffrey Martin, Hawkins Stephen A.C., Dawson Michael, Hope James, High prevalence of scrapie in a dairy goat herd: tissue distribution of disease-associated PrP and effect ofPRNPgenotype and age, 10.1051/vetres/2009048
Gonzalez L., Siso S., Monleon E., Casalone C., van Keulen L. J. M., Balkema-Buschmann A., Ortiz-Pelaez A., Iulini B., Langeveld J. P. M., Hoffmann C., Badiola J. J., Jeffrey M., Acin C., Variability in disease phenotypes within a single PRNP genotype suggests the existence of multiple natural sheep scrapie strains within Europe, 10.1099/vir.0.022574-0
González Lorenzo, Jeffrey Martin, Dagleish Mark P, Goldmann Wilfred, Sisó Sílvia, Eaton Samantha L, Martin Stuart, Finlayson Jeanie, Stewart Paula, Steele Philip, Pang Yvonne, Hamilton Scott, Reid Hugh W, Chianini Francesca, Susceptibility to scrapie and disease phenotype in sheep: cross-PRNP genotype experimental transmissions with natural sources, 10.1186/1297-9716-43-55
González L., Pitarch J.L., Martin S., Thurston L., Simmons H., Acín C., Jeffrey M., Influence of Polymorphisms in the Prion Protein Gene on the Pathogenesis and Neuropathological Phenotype of Sheep Scrapie after Oral Infection, 10.1016/j.jcpa.2013.10.001
Gossner A.G., Bennet N., Hunter N., Hopkins J., Differential expression of Prnp and Sprn in scrapie infected sheep also reveals Prnp genotype specific differences, 10.1016/j.bbrc.2008.12.002
Green K. M., Browning S. R., Seward T. S., Jewell J. E., Ross D. L., Green M. A., Williams E. S., Hoover E. A., Telling G. C., The elk PRNP codon 132 polymorphism controls cervid and scrapie prion propagation, 10.1099/vir.0.83168-0
Griffiths P. C., Spiropoulos J., Lockey R., Tout A. C., Jayasena D., Plater J. M., Chave A., Green R. B., Simonini S., Thorne L., Dexter I., Balkema-Buschmann A., Groschup M. H., Beringue V., Le Dur A., Laude H., Hope J., Characterization of atypical scrapie cases from Great Britain in transgenic ovine PrP mice, 10.1099/vir.0.018986-0
Groschup Martin H., Buschmann Anne, Rodent models for prion diseases, 10.1051/vetres:2008008
Groschup Martin H., Lacroux Caroline, Buschmann Anne, Lühken Gesine, Mathey Jacinthe, Eiden Martin, Lugan Séverine, Hoffmann Christine, Espinosa Juan Carlos, Baron Thierry, Torres Juan Maria, Erhardt Georg, Andreoletti Olivier, Classic Scrapie in Sheep with the ARR/ARR Prion Genotype in Germany and France, 10.3201/eid1308.070077
Gubbins Simon, Cook Charlotte J, Hyder Kieran, Boulton Kay, Davis Carol, Thomas Eurion, Haresign Will, Bishop Stephen C, Villanueva Beatriz, Eglin Rachel D, Associations between lamb survival and prion protein genotype: analysis of data for ten sheep breeds in Great Britain, 10.1186/1746-6148-5-3
Hadlow W. J., Kennedy R. C., Race R. E., Eklund C. M., Virologic and Neurohistologic Findings in Dairy Goats Affected with Natural Scrapie, 10.1177/030098588001700207
Healy Anne M., Hannon Deirdre, Morgan Kenton L., Weavers Edwin, Dan Collins J., Doherty Michael L., A paired case–control study of risk factors for scrapie in Irish sheep flocks, 10.1016/j.prevetmed.2004.06.002
Hoinville Linda J., Tongue Susan C., Wilesmith John W., Evidence for maternal transmission of scrapie in naturally affected flocks, 10.1016/j.prevetmed.2009.10.013
Hourrigan, Slow Transmissible Diseases of the Nervous System, 1, 331 (1979)
Houston Fiona, Goldmann Wilfred, Chong Angela, Jeffrey Martin, González Lorenzo, Foster James, Parnham David, Hunter Nora, Prion diseases: BSE in sheep bred for resistance to infection, 10.1038/423498a
Houston Fiona, Goldmann Wilfred, Foster James, González Lorenzo, Jeffrey Martin, Hunter Nora, Comparative Susceptibility of Sheep of Different Origins, Breeds and PRNP Genotypes to Challenge with Bovine Spongiform Encephalopathy and Scrapie, 10.1371/journal.pone.0143251
Hunter N., Goldmann W., Benson G., Foster J. D., Hope J., Swaledale sheep affected by natural scrapie differ significantly in PrP genotype frequencies from healthy sheep and those selected for reduced incidence of scrapie, 10.1099/0022-1317-74-6-1025
Hunter N., Foster J. D., Goldmann W., Stear M. J., Hope J., Bostock C., Natural scrapie in a closed flock of Cheviot sheep occurs only in specific PrP genotypes, 10.1007/bf01718157
Hunter N., Goldmann W., Foster J. D., Cairns D., Smith G., Natural scrapie and PrP genotype: case-control studies in British sheep, 10.1136/vr.141.6.137
Hunter N., Houston F., Foster J., Goldmann W., Drummond D., Parnham D., Kennedy I., Green A., Stewart P., Chong A., Susceptibility of Young Sheep to Oral Infection with Bovine Spongiform Encephalopathy Decreases Significantly after Weaning, 10.1128/jvi.01573-12
Huybrechts Inge, Sioen Isabelle, Boon Polly E, Ruprich Jiri, Lafay Lionel, Turrini Aida, Amiano Pilar, Hirvonen Tero, De Neve Melissa, Arcella Davide, Moschandreas Joanna, Westerlund Anna, Ribas-Barba Lourdes, Hilbig Annett, Papoutsou Stalo, Christensen Tue, Oltarzewski Maciej, Virtanen Suvi, Rehurkova Irena, Azpiri Mikel, Sette Stefania, Kersting Mathilde, Walkiewicz Alicja, Serra-Majem Luis, Volatier Jean-Luc, Trolle Ellen, Tornaritis Michael, Busk Leif, Kafatos Anthony, Fabiansson Stefan, De Henauw Stefaan, Van Klaveren Jacob D, Dietary exposure assessments for children in europe (the EXPOCHI project): rationale, methods and design, 10.1186/0778-7367-69-4
Jacobs J. G., Sauer M., van Keulen L. J. M., Tang Y., Bossers A., Langeveld J. P. M., Differentiation of ruminant transmissible spongiform encephalopathy isolate types, including bovine spongiform encephalopathy and CH1641 scrapie, 10.1099/vir.0.026153-0
Jacobs J. G., Bossers A., Rezaei H., van Keulen L. J. M., McCutcheon S., Sklaviadis T., Lantier I., Berthon P., Lantier F., van Zijderveld F. G., Langeveld J. P. M., Proteinase K-Resistant Material in ARR/VRQ Sheep Brain Affected with Classical Scrapie Is Composed Mainly of VRQ Prion Protein, 10.1128/jvi.00448-11
Jeffrey M., Ryder S., Martin S., Hawkins S.A.C., Terry L., Berthelin-Baker C., Bellworthy S.J., Oral Inoculation of Sheep with the Agent of Bovine Spongiform Encephalopathy (BSE). 1. Onset and Distribution of Disease-specific PrP Accumulation in Brain and Viscera, 10.1053/jcpa.2001.0465
Jeffrey M., Martin S., González L., Foster J., Langeveld J.P.M., van Zijderveld F.G., Grassi J., Hunter N., Immunohistochemical Features of PrPd Accumulation in Natural and Experimental Goat Transmissible Spongiform Encephalopathies, 10.1016/j.jcpa.2005.10.003
Johnson Chad J., Herbst Allen, Duque-Velasquez Camilo, Vanderloo Joshua P., Bochsler Phil, Chappell Rick, McKenzie Debbie, Prion Protein Polymorphisms Affect Chronic Wasting Disease Progression, 10.1371/journal.pone.0017450
Kanata Eirini, Humphreys-Panagiotidis Cynthia, Giadinis Nektarios D, Papaioannou Nikolaos, Arsenakis Minas, Sklaviadis Theodoros, Perspectives of a scrapie resistance breeding scheme targeting Q211, S146 and K222 caprine PRNP alleles in Greek goats, 10.1186/1297-9716-45-43
Kanata Eirini, Arsenakis Minas, Sklaviadis Theodoros, Caprine PrP variants harboring Asp-146, His-154 and Gln-211 alleles display reduced convertibility upon interaction with pathogenic murine prion protein in scrapie infected cells, 10.1080/19336896.2016.1199312
Kimberlin R. H., Walker C. A., Incubation Periods in Six Models of Intraperitoneally Injected Scrapie Depend Mainly on the Dynamics of Agent Replication within the Nervous System and Not the Lymphoreticular System, 10.1099/0022-1317-69-12-2953
Konold Timm, Bone Gemma E, Phelan Laura J, Simmons Marion M, Gonzalez Lorenzo, Siso Silvia, Goldmann Wilfred, Cawthraw Saira, Hawkins Steve AC, Monitoring of clinical signs in goats with transmissible spongiform encephalopathies, 10.1186/1746-6148-6-13
Konold Timm, Simmons Hugh A., Webb Paul R., Bellerby Peter J., Hawkins Steve A. C., González Lorenzo, Transmission of classical scrapie via goat milk, 10.1136/vr.f2613
Konold Timm, Thorne Leigh, Simmons Hugh A., Hawkins Steve A. C., Simmons Marion M., González Lorenzo, Evidence of scrapie transmission to sheep via goat milk, 10.1186/s12917-016-0807-4
Lacroux C., Perrin-Chauvineau C., Corbiere F., Aron N., Aguilar-Calvo P., Torres J. M., Costes P., Bremaud I., Lugan S., Schelcher F., Barillet F., Andreoletti O., Caughey B. W., Genetic Resistance to Scrapie Infection in Experimentally Challenged Goats, 10.1128/jvi.02872-13
Lacroux C, Cassard H, Simmons H, Douet JY, Corbière F, Lugan S, Coste P, Aron N, Huor A, Tillier C, Schelcher F and Andreoletti O, in press. Classical scrapie transmission in ARR/ARR genotype sheep.
Lampo Evelyne, Van Poucke Mario, Hugot Karine, Hayes Hélène, Van Zeveren Alex, Peelman Luc J, Characterization of the genomic region containing the Shadow of Prion Protein (SPRN) gene in sheep, 10.1186/1471-2164-8-138
Langeveld J. P. M., Jacobs J. G., Hunter N., van Keulen L. J. M., Lantier F., van Zijderveld F. G., Bossers A., Prion Type-Dependent Deposition ofPRNPAllelic Products in Heterozygous Sheep, 10.1128/jvi.02316-15
Le Dur A., Beringue V., Andreoletti O., Reine F., Lai T. L., Baron T., Bratberg B., Vilotte J.-L., Sarradin P., Benestad S. L., Laude H., A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes, 10.1073/pnas.0502296102
Maestrale Caterina, Cancedda Maria G., Pintus Davide, Masia Mariangela, Nonno Romolo, Ru Giuseppe, Carta Antonello, Demontis Francesca, Santucciu Cinzia, Ligios Ciriaco, Genetic and Pathological Follow-Up Study of Goats Experimentally and Naturally Exposed to a Sheep Scrapie Isolate, 10.1128/jvi.01262-15
Manson J. C., Clarke A. R., Hooper M. L., Aitchison L., McConnell I., Hope J., 129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal, 10.1007/bf02780662
McIntyre K. Marie, Gubbins Simon, Goldmann Wilfred, Hunter Nora, Baylis Matthew, Epidemiological Characteristics of Classical Scrapie Outbreaks in 30 Sheep Flocks in the United Kingdom, 10.1371/journal.pone.0003994
Mead Simon, Joiner Susan, Desbruslais Melanie, Beck Jonathan A., O’Donoghue Michael, Lantos Peter, Wadsworth Jonathan D. F., Collinge John, Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel PrPSc Type in a Young British Woman, 10.1001/archneur.64.12.1780
Meade-White K., Race B., Trifilo M., Bossers A., Favara C., Lacasse R., Miller M., Williams E., Oldstone M., Race R., Chesebro B., Resistance to Chronic Wasting Disease in Transgenic Mice Expressing a Naturally Occurring Allelic Variant of Deer Prion Protein, 10.1128/jvi.02762-06
Merten C., Ferrari P., Bakker M., Boss A., Hearty Á., Leclercq C., Lindtner O., Tlustos C., Verger P., Volatier J.L., Arcella D., Methodological characteristics of the national dietary surveys carried out in the European Union as included in the European Food Safety Authority (EFSA) Comprehensive European Food Consumption Database, 10.1080/19440049.2011.576440
Moore Richard C, Lee Inyoul Y, Silverman Gregory L, Harrison Paul M, Strome Robert, Heinrich Cornelia, Karunaratne Amila, Pasternak Stephen H, Chishti M.Azhar, Liang Yan, Mastrangelo Peter, Wang Kai, Smit Arian F.A, Katamine Shigeru, Carlson George A, Cohen Fred E, Prusiner Stanley B, Melton David W, Tremblay Patrick, Hood Leroy E, Westaway David, Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel, 10.1006/jmbi.1999.3108
Moreno C. R., Moazami-Goudarzi K., Laurent P., Cazeau G., Andreoletti O., Chadi S., Elsen J.-M., Calavas D., Which PrP haplotypes in a French sheep population are the most susceptible to atypical scrapie?, 10.1007/s00705-007-0956-7
Niedermeyer Susanne, Eiden Martin, Toumazos Pavlos, Papasavva-Stylianou Penelope, Ioannou Ioannis, Sklaviadis Theodoros, Panagiotidis Cynthia, Langeveld Jan, Bossers Alex, Kuczius Thorsten, Kaatz Martin, Groschup Martin H., Fast Christine, Genetic, histochemical and biochemical studies on goat TSE cases from Cyprus, 10.1186/s13567-016-0379-0
OKADA Hiroyuki, MIYAZAWA Kohtaro, MASUJIN Kentaro, YOKOYAMA Takashi, Coexistence of two forms of disease-associated prion protein in extracerebral tissues of cattle infected with H-type bovine spongiform encephalopathy, 10.1292/jvms.16-0068
O'Rourke K I, Besser T E, Melco R P, Foote W C, Clark W W, Wang S, Holyoak G R, Mickelson J R, PrP genotypes and experimental scrapie in orally inoculated Suffolk sheep in the United States., 10.1099/0022-1317-78-4-975
O'Rourke Katherine I, Zhuang Dongyue, Truscott Thomas C, Yan Huijan, Schneider David A, Sparse PrPSc accumulation in the placentas of goats with naturally acquired scrapie, 10.1186/1746-6148-7-7
Ortiz-Pelaez Angel, Bianchini Juana, The impact of the genotype on the prevalence of classical scrapie at population level, 10.1186/1297-9716-42-31
Ortiz-Pelaez A., Kelly L., Adkin A., The risk of introducing scrapie from restocking goats in Great Britain, 10.1016/j.prevetmed.2012.05.018
ORTIZ-PELAEZ A., GEORGIADOU S., SIMMONS M. M., WINDL O., DAWSON M., ARNOLD M. E., NEOCLEOUS P., PAPASAVVA-STYLIANOU P., Allelic variants at codon 146 in the PRNP gene show significant differences in the risk for natural scrapie in Cypriot goats, 10.1017/s0950268814002064
Papasavva-Stylianou Penelope, Kleanthous Marina, Toumazos Pavlos, Mavrikiou Petroula, Loucaides Phedias, Novel polymorphisms at codons 146 and 151 in the prion protein gene of Cyprus goats, and their association with natural scrapie, 10.1016/j.tvjl.2005.09.013
Papasavva-Stylianou Penelope, Windl Otto, Saunders Ginny, Mavrikiou Petroula, Toumazos Pavlos, Kakoyiannis Charalambos, PrP gene polymorphisms in Cyprus goats and their association with resistance or susceptibility to natural scrapie, 10.1016/j.tvjl.2009.10.015
Pattison I.H., Millson G.C., Scrapie Produced Experimentally in Goats With Special Reference To the Clinical Syndrome, 10.1016/s0368-1742(61)80013-1
Pattison I., Hoare M., Jebbett J., Watson W., Spread of scrapie to sheep and goats by oral dosing with foetal membranes from scrapie-affected sheep, 10.1136/vr.90.17.465
Peletto S., Bertolini S., Maniaci M. G., Colussi S., Modesto P., Biolatti C., Bertuzzi S., Caramelli M., Maurella C., Acutis P. L., Association of an indel polymorphism in the 3'UTR of the caprine SPRN gene with scrapie positivity in the central nervous system, 10.1099/vir.0.041400-0
Pirisinu Laura, Marcon Stefano, Bari Michele, D'Agostino Claudia, Agrimi Umberto, Nonno Romolo, Biochemical Characterization of Prion Strains in Bank Voles, 10.3390/pathogens2030446
Priem J., Langeveld J. P. M., van Keulen L. J. M., van Zijderveld F. G., Andreoletti O., Bossers A., Enhanced Virulence of Sheep-Passaged Bovine Spongiform Encephalopathy Agent Is Revealed by Decreased Polymorphism Barriers in Prion Protein Conversion Studies, 10.1128/jvi.02446-13
Pritchard T. C., Cahalan C. M., Ap Dewi I., Association between PrP genotypes and performance traits in a Welsh Mountain flock, 10.1017/s1751731108002747
Ryder S, Dexter G, Bellworthy S, Tongue S, Demonstration of lateral transmission of scrapie between sheep kept under natural conditions using lymphoid tissue biopsy, 10.1016/j.rvsc.2003.11.007
Saborio Gabriela P., Permanne Bruno, Soto Claudio, Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding, 10.1038/35081095
Sawalha R. M., Villanueva B., Brotherstone S., Rogers P. L., Lewis R. M., Prediction of prion protein genotype and association of this genotype with lamb performance traits of Suffolk sheep, 10.2527/jas.2009-2009
Schneider David A., Madsen-Bouterse Sally A., Truscott Thomas C., Zhuang Dongyue, O'Rourke Katherine I., Dassanayake Rohana P., The placenta shed from goats with classical scrapie is infectious to goat kids and lambs, 10.1099/vir.0.000151
Seidel Bjoern, Thomzig Achim, Buschmann Anne, Groschup Martin H., Peters Rainer, Beekes Michael, Terytze Konstantin, Scrapie Agent (Strain 263K) Can Transmit Disease via the Oral Route after Persistence in Soil over Years, 10.1371/journal.pone.0000435
Seuberlich Torsten, Botteron Catherine, Benestad Sylvie L., Brünisholz Hervé, Wyss Reto, Kihm Ulrich, Schwermer Heinzpeter, Friess Martina, Nicolier Alexandra, Heim Dagmar, Zurbriggen Andreas, Atypical Scrapie in a Swiss Goat and Implications for Transmissible Spongiform Encephalopathy Surveillance, 10.1177/104063870701900102
Simmons Marion M., Moore S. Jo, Konold Timm, Thurston Lisa, Terry Linda A., Thorne Leigh, Lockey Richard, Vickery Chris, Hawkins Stephen A.C., Chaplin Melanie J., Spiropoulos John, Experimental Oral Transmission of Atypical Scrapie to Sheep, 10.3201/eid1705.101654
Simmons Marion M., Moore S. Jo, Lockey Richard, Chaplin Melanie J, Konold Timm, Vickery Christopher, Spiropoulos John, Phenotype Shift from Atypical Scrapie to CH1641 following Experimental Transmission in Sheep, 10.1371/journal.pone.0117063
Sofianidis G., Psychas V., Billinis C., Spyrou V., Argyroudis S., Papaioannou N., Vlemmas I., Histopathological and Immunohistochemical Features of Natural Goat Scrapie, 10.1016/j.jcpa.2006.06.004
Soto Claudio, Saborio Gabriela P, Anderes Laurence, Cyclic amplification of protein misfolding: application to prion-related disorders and beyond, 10.1016/s0166-2236(02)02195-1
Spiropoulos John, Lockey Richard, Sallis Rosemary E., Terry Linda A., Thorne Leigh, Holder Thomas M., Beck Katy E., Simmons Marion M., Isolation of Prion with BSE Properties from Farmed Goat, 10.3201/eid1712.110333
Srithayakumar Vythegi, Mitchell Gordon B., White Bradley N., Identification of amino acid variation in the prion protein associated with classical scrapie in Canadian dairy goats, 10.1186/s12917-016-0684-x
SSC , 1999. Scientific Opinion on the policy of breeding and genotyping of sheep on the policy of breeding and genotyping of sheep, i.e. the issue of whether sheep should be bred to be resistant to scrapie, adopted by the Scientific Steering Committee at its meeting of 22–23 July 1999.
SSC , 2002. Opinion on safe sourcing of small ruminant materials (safe sourcing of small ruminant materials should BSE in small ruminants become probable: genotype, breeding, rapid TSE testing, flocks certification and specified risk materials), adopted by the Scientific Steering Committee at its meeting of 4–5 April 2002.
Stamp, The Veterinary Record, 74, 357 (1962)
Stewart P., Shen C., Zhao D., Goldmann W., Genetic analysis of the SPRN gene in ruminants reveals polymorphisms in the alanine-rich segment of shadoo protein, 10.1099/vir.0.011494-0
Tabouret G., Lacroux C., Lugan S., Costes P., Corbiere F., Weisbecker J. L., Schelcher F., Andreoletti O., Relevance of oral experimental challenge with classical scrapie in sheep, 10.1099/vir.0.021311-0
Taema Maged M., Maddison Ben C., Thorne Leigh, Bishop Keith, Owen Jonathan, Hunter Nora, Baker Claire A., Terry Linda A., Gough Kevin C., Differentiating Ovine BSE from CH1641 Scrapie by Serial Protein Misfolding Cyclic Amplification, 10.1007/s12033-011-9460-0
Thackray A. M., Hopkins L., Lockey R., Spiropoulos J., Bujdoso R., Emergence of multiple prion strains from single isolates of ovine scrapie, 10.1099/vir.0.028886-0
Thackray A.M., Lockey R., Beck K.E., Spiropoulos J., Bujdoso R., Evidence for Co-infection of Ovine Prion Strains in Classical Scrapie Isolates, 10.1016/j.jcpa.2012.01.009
Thorne Leigh, Holder Thomas, Ramsay Andrew, Edwards Jane, Taema Maged, Windl Otto, Maddison Ben Charles, Gough Kevin Christopher, Terry Linda Ann, In vitro amplification of ovine prions from scrapie-infected sheep from Great Britain reveals distinct patterns of propagation, 10.1186/1746-6148-8-223
Tuzi Nadia L., Manson Jean C., Melton David, Gall Elaine, Expression of doppel in the CNS of mice does not modulate transmissible spongiform encephalopathy disease, 10.1099/0022-1317-83-3-705
Vaccari G., Petraroli R., Agrimi U., Eleni C., Perfetti M. G., Di Bari M. A., Morelli L., Ligios C., Busani L., Nonno R., Di Guardo G., PrP genotype in Sarda breed sheep and its relevance to scrapie, 10.1007/s007050170050
Vaccari G., Identification of an allelic variant of the goat PrP gene associated with resistance to scrapie, 10.1099/vir.0.81485-0
Vaccari Gabriele, Scavia Gaia, Sala Marcello, Cosseddu Gianmario, Chiappini Barbara, Conte Michela, Esposito Elena, Lorenzetti Raniero, Perfetti Gabriella, Marconi Paola, Scholl Francesco, Barbaro Katia, Bella Antonino, Nonno Romolo, Agrimi Umberto, Protective effect of the AT137RQ and ARQK176PrP allele against classical scrapie in Sarda breed sheep, 10.1051/vetres/2009002
Vilotte J.-L., Soulier S., Essalmani R., Stinnakre M.-G., Vaiman D., Lepourry L., Da Silva J. C., Besnard N., Dawson M., Buschmann A., Groschup M., Petit S., Madelaine M.-F., Rakatobe S., Le Dur A., Vilette D., Laude H., Markedly Increased Susceptibility to Natural Sheep Scrapie of Transgenic Mice Expressing Ovine PrP, 10.1128/jvi.75.13.5977-5984.2001
Vitale Maria, Migliore Sergio, La Giglia Maria, Alberti Placido, Di Marco Lo Presti Vincenzo, Langeveld Jan P. M., Scrapie incidence and PRNP polymorphisms: rare small ruminant breeds of Sicily with TSE protecting genetic reservoirs, 10.1186/s12917-016-0766-9
Wadsworth J. D. F., Asante E. A., Collinge J., Review: Contribution of transgenic models to understanding human prion disease : Transgenic models of human prion disease, 10.1111/j.1365-2990.2010.01129.x
Wang Haiying, Wan Jiayu, Wang Wei, Wang Dongxu, Li Sha, Liao Peng, Hao Zhuo, Wu Songbo, Xu Jing, Li Nan, OuYang Hongsheng, Gao Hongwei, Overexpression of Shadoo protein in transgenic mice does not impact the pathogenesis of scrapie, 10.1016/j.neulet.2011.03.073
Watts Joel C., Westaway David, The prion protein family: Diversity, rivalry, and dysfunction, 10.1016/j.bbadis.2007.05.001
Watts Joel C., Stöhr Jan, Bhardwaj Sumita, Wille Holger, Oehler Abby, DeArmond Stephen J., Giles Kurt, Prusiner Stanley B., Protease-Resistant Prions Selectively Decrease Shadoo Protein, 10.1371/journal.ppat.1002382
Westaway David, Goodman Patricia A., Mirenda Carol A., McKinley Michael P., Carlson George A., Prusiner Stanley B., Distinct prion proteins in short and long scrapie incubation period mice, 10.1016/0092-8674(87)90134-6
White Stephen N., Reynolds James O., Waldron Daniel F., Schneider David A., O'Rourke Katherine I., Extended scrapie incubation time in goats singly heterozygous for PRNP S146 or K222, 10.1016/j.gene.2012.03.068
Windig, Animal Science, 79, 11 (2004)
Windig J.J., Hoving R.A.H., Priem J., Bossers A., van Keulen L.J.M., Langeveld J.P.M., Variation in the prion protein sequence in Dutch goat breeds, 10.1111/jbg.12211
Zlotnik I., Rennie J.C., Further observations on the experimental transmission of scrapie from sheep and goats to laboratory mice, 10.1016/s0368-1742(63)80018-1
Bibliographic reference
EFSA Panel on Biological Hazards (BIOHAZ) ; Ricci, Antonia ; Allende, Ana ; Bolton, Declan ; Chemaly, Marianne ; et. al. Genetic resistance to transmissible spongiform encephalopathies (TSE) in goats. In: EFSA Journal, Vol. 15, no. 8, p. 4962 [1-79] (2017)