OMIA:000944-9925 : Spongiform encephalopathy, susceptibility/resistance to in Capra hircus (goat)

In other species: Mallard , chicken , Ring-necked pheasant , white-tufted-ear marmoset , macaques , crab-eating macaque , Rhesus monkey , dog , domestic ferret , domestic cat , puma , horse , pig , Arabian camel , deer , Eurasian elk , Western roe deer , red deer , Eastern wapiti , sika deer , Manchurian Wapiti , reindeer , black-tailed deer , white-tailed deer , American bison , taurine cattle , mouflon , sheep , eland , greater kudu , gemsbok , rabbit , golden hamster , domestic guinea pig , domestic yak , chital , fallow deer , cheetah , raccoon dog , bighorn sheep , blue antelope , Arabian oryx , scimitar-horned oryx , nyala , Spanish ibex , water buffalo , Japanese quail , Pyrenean chamois , Iberian red deer , Bank vole , American mink

Categories: Nervous system phene

Links to possible relevant human trait(s) and/or gene(s) in OMIM: 176640 (gene) , 245300 (trait)

Links to relevant human diseases in MONDO:

Mendelian trait/disorder: unknown

Considered a defect: yes

Year key variant first reported: 1996

Cross-species summary: Spongiform encephalopathies are a class of fatal neurological diseases. Clinical signs are characteristic of a progressive degeneration of the central nervous system; they include pruritis, abnormalities of gait and recumbency. Death is inevitable. On post-mortem, brain histopathology shows a characteristic spongy appearance. The infectious agent is a modified form of a protein encoded by a gene in the host. The name given to this infectious particle is prion. The host gene is called the prion protein (PrP) gene, which is a normal part of the genome of mammals and chickens. Its polypeptide product, called cellular PrP(superscript C), is a naturally-occurring protein attached to the outer surface of neurones and some other cells. PrP(superscript C) appears to play a role in maintaining the Purkinje cells of the cerebellum, which are essential for balance and muscular function. The infectious agent, called scrapie PrP(superscript Sc), is a modifed form of PrP(superscript C), where the modifications involve glycosylation and the creation of intra-strand di-sulphide bonds. It is important to realise that these modifications involve no change in amino acid sequence. When PrP(superscript Sc) molecules enter a previously uninfected host, they convert the naturally occurring PrP(superscript C) molecules, produced by the host gene, into infectious PrP(superscript Sc) particles, which ultimately cause clinical signs in that animal, and which can spread to other animals, both horizontally (by infection) and vertically (by maternal transmission). In ruminants the disease has been called bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep and goats and chronic wasting disease (CWD) in cervids.

Species-specific name: Scrapie, calssical; scrapie, atypical

Species-specific description: A review of classical and atypical scrapie in sheep and goat is provided by Acin et al. (2021). The European Food Safety Authority (EFSA) panel on biological hazards (2017) reviewed information relating to genetic resistance of transmissible spongiform encephalopathies in goats and developed a tool to "evaluate the genetic resistance to classical scrapie of alleles in the goat PRNP gene, which considers both the weight of evidence and strength of resistance for each allele of interest."

Molecular basis: Gelasakis et al. (2021): in goats “more than 50 PRNP gene polymorphisms have been found, however, their role regarding the incubation period and the susceptibility to classical scrapie has not been adequately documented.” Goldmann et al. (1996) reported a goat PRNP variant that is "identical to the most common sheep PrP protein variant (Ala136-Arg154-Gln171). The dimorphism at codon 142 (Ile --> Met) appeared to be associated with differing disease incubation periods in goats experimentally infected with isolates of bovine spongiform encephalopathy, sheep scrapie CH1641 or sheep-passaged ME7 scrapie." Vaccari et al. (2006) reported an "allelic variant encoding lysine at position 222 could be a valuable candidate to select in the framework of appropriate breeding programmes for scrapie resistance in goats." Papasavva-Stylianou et al. (2007) reported "novel amino acid polymorphisms were detected at [codon] 146 (N-->S or D) . . . The two novel polymorphisms at codon 146 were found only in the healthy control and scrapie-negative goats. By comparison, none of the scrapie-affected goats encoded these polymorphisms." Benistad et al. (2012) reported a nonsense variant (now called Ter) in codon 32 of the PRNP gene that results in "Healthy [Norwegian dairy] goats naturally devoid of prion protein". The authors predicted that these goats would be "resistant to prion disease" because they completely lack the prion protein which, when misfolded, is causative of spongiform encephalopathy. In a comprehensive comparison of +/+, +/Ter and Ter/Ter goats, Salvesen et al. (2020) showed "that goats naturally devoid of PrPC do not replicate prions and are therefore resistant to prion disease. The onset of disease and the progression of clinical signs is delayed in heterozygous animals. Pathological changes and the distribution of PrPSc in the CNS were similar in PRNP+/+ and PRNP+/Ter goats, and there was only a limited peripheral spread of PrPSc to head lymph nodes and nervous tissue in close proximity to the CNS." Gelasakis et al. (2021) investigated “brain samples (n = 525) from scrapie-affected (n = 282) and non-affected (n = 243) animals within the national surveillance program, and (ii) individual blood samples (n = 1708) from affected (n = 241) and non-affected (n = 1467) herds, in a large part of mainland Greece and its islands … . A dedicated Taqman method was used to test for amino acid polymorphisms 110T/P, 146N/S/D, 211R/Q, and 222Q/K.” The authors concluded that "breeding programs in Karpathos could focus on codon 146D, while in other regions carriers of the 110P and 222K allele should be sought.” Adeola et al. (2023) "evaluated the genetic and allele distributions of PRNP polymorphism in 132 Nigerian goats and compared them with publicly available studies on scrapie-affected goats. ... Our study revealed 29 SNPs in Nigerian goats, of which 14 were non-synonymous, and 23 were novel. There were significant differences (P < 0.001) in the allele frequencies of PRNP codons 139, 146, 154 and 193 in Nigerian goats compared with scrapie-affected goats, except for Northern Italian goats at codon 154."

Genetic testing: Sacchi et al. (2018): "The European Union has implemented breeding programmes to increase scrapie resistance in sheep. A similar approach can be applied also in goats since the K222 allele provides a level of resistance equivalent to that of ARR in sheep. The European Food Safety Authority stated that breeding for resistance could be offered as an option for Member States to control classical scrapie in goats." EFSA panel on biological hazards (2017) "The K222, D146 and S146 alleles confer genetic resistance to classical scrapie strains known to occur in the EU goat population. The K222 polymorphism confers resistance against a variety of EU classical scrapie isolates that may reflect a variety of scrapie strains. However, there is no assurance that K222 carriers would be resistant to all TSE agent strains currently circulating in the EU goat population. The D146 and S146 alleles are associated with strong resistance against scrapie agent(s) currently circulating in Cyprus. However, data remain insufficient to assess the level of resistance that D146 and S146 might provide against other classical scrapie agents circulating in other EU goat populations. The weight of evidence for K222 is greater than that currently available for the D146 and S146 alleles and for the ARR allele in sheep at the time the 2002 SSC opinion was produced. ... Due to insufficient data, it is not possible to provide a ranking of susceptibility at genotype level. Based on a combination of the ‘weight of evidence’ and the ‘strength of resistance’, alleles can currently be ranked as follows, from high to low classical scrapie resistance: K222 > D146 = S146 >Q211 = H154 = M142 > S127 = H143 > wild type. ... There is extremely limited data on the susceptibility of goats to either atypical scrapie or BSE for the nine alleles of interest. For atypical scrapie, it was hypothesised that the H154 allele is likely to be associated with higher risk of developing atypical scrapie. For BSE, there are currently no data available on the resistance/susceptibility to BSE infection in D146 and S146 allele carriers, but an oral challenge study indicated incomplete resistance to BSE in K222 allele carriers. There are no data available on the susceptibility of goats with the H154 allele to BSE infection."

Associated gene:

Symbol Description Species Chr Location OMIA gene details page Other Links
PRNP prion protein Capra hircus 13 NC_030820.1 (46453052..46473740) PRNP Homologene, Ensembl , NCBI gene


By default, variants are sorted chronologically by year of publication, to provide a historical perspective. Readers can re-sort on any column by clicking on the column header. Click it again to sort in a descending order. To create a multiple-field sort, hold down Shift while clicking on the second, third etc relevant column headers.

WARNING! Inclusion of a variant in this table does not automatically mean that it should be used for DNA testing. Anyone contemplating the use of any of these variants for DNA testing should examine critically the relevant evidence (especially in breeds other than the breed in which the variant was first described). If it is decided to proceed, the location and orientation of the variant sequence should be checked very carefully.

Since October 2021, OMIA includes a semiautomated lift-over pipeline to facilitate updates of genomic positions to a recent reference genome position. These changes to genomic positions are not always reflected in the ‘acknowledgements’ or ‘verbal description’ fields in this table.

OMIA Variant ID Breed(s) Variant Phenotype Gene Allele Type of Variant Source of Genetic Variant Reference Sequence Chr. g. or m. c. or n. p. Verbal Description EVA ID Year Published PubMed ID(s) Acknowledgements
1310 Resistance to scrapie PRNP missense Naturally occurring variant ARS1 13 p.(H143R) 2002 11842266
1146 Norsk melkegeit, Norway (Goat) Complete resistance to scrapie PRNP Ter nonsense (stop-gain) Naturally occurring variant ARS1 13 g.46469788G>T c.94G>T p.(G32*) ENSEMBL Variant Effect Predictor: transcript ENSCHIT00000020215.1 2012 23249298 The genomic location on ARS1 was determined by Katie Eager, EMAI, NSW Department of Primary Industries
827 Resistance to scrapie PRNP missense Naturally occurring variant ARS1 13 g.46470073G>A c.379G>A p.(G127S) ENSEMBL Variant Effect Predictor: transcript ENSCHIT00000020215.1 rs268292978 2011 22040234 The genomic location on ARS1 was determined by Katie Eager, EMAI, NSW Department of Primary Industries
828 Resistance to scrapie PRNP missense Naturally occurring variant ARS1 13 g.46470120A>G c.426A>G p.(I142M) ENSEMBL Variant Effect Predictor: transcript ENSCHIT00000020215.1 rs268292980 1996 8922485 The genomic location on ARS1 was determined by Katie Eager, EMAI, NSW Department of Primary Industries
830 Resistance to scrapie PRNP missense Naturally occurring variant ARS1 13 g.46470130A>G c.436A>G p.(N146D) ENSEMBL Variant Effect Predictor: transcript ENSCHIT00000020215.1 2007 16314132 The genomic location on ARS1 was determined by Katie Eager, EMAI, NSW Department of Primary Industries
829 Resistance to scrapie PRNP missense Naturally occurring variant ARS1 13 g.46470131A>G c.437A>G p.(N146S) ENSEMBL Variant Effect Predictor: transcript ENSCHIT00000020215.1 2007 16314132 The genomic location on ARS1 was determined by Katie Eager, EMAI, NSW Department of Primary Industries
831 Resistance to scrapie PRNP missense Naturally occurring variant ARS1 13 g.46470155G>A c.461G>A p.(R154H) ENSEMBL Variant Effect Predictor: transcript ENSCHIT00000020215.1 rs268292981 2009 19218225 The genomic location on ARS1 was determined by Katie Eager, EMAI, NSW Department of Primary Industries
832 Resistance to scrapie PRNP missense Naturally occurring variant ARS1 13 g.46470326G>A c.632G>A p.(R211Q) ENSEMBL Variant Effect Predictor: transcript ENSCHIT00000020215.1 rs268292982 2009 19218225 The genomic location on ARS1 was determined by Katie Eager, EMAI, NSW Department of Primary Industries
833 Resistance to scrapie PRNP missense Naturally occurring variant ARS1 13 g.46470358C>A c.664C>A p.(Q222K) ENSEMBL Variant Effect Predictor: transcript ENSCHIT00000020215.1 rs268292983 2006 16603543 The genomic location on ARS1 was determined by Katie Eager, EMAI, NSW Department of Primary Industries

Cite this entry

Nicholas, F. W., Tammen, I., & Sydney Informatics Hub. (2023). OMIA:000944-9925: Online Mendelian Inheritance in Animals (OMIA) [dataset].


Note: the references are listed in reverse chronological order (from the most recent year to the earliest year), and alphabetically by first author within a year.

2024 Adeola, A.C., Bello, S.F., Abdussamad, A.M., Adedokun, R.A.M., Olaogun, S.C., Abdullahi, N., Mark, A.I., Onoja, A.B., Sanke, O.J., Mangbon, G.F., Ibrahim, J., Dawuda, P.M., Salako, A.E., Kdidi, S., Yahyaoui, M.H. :
Single nucleotide polymorphisms (SNPs) in the open reading frame (ORF) of prion protein gene (PRNP) in Nigerian livestock species. BMC Genomics 25:177, 2024. Pubmed reference: 38355406. DOI: 10.1186/s12864-024-10070-2.
2023 [No authors listed] :
The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2022. EFSA J 21:e08384, 2023. Pubmed reference: 38035139. DOI: 10.2903/j.efsa.2023.8384.
Adeola, A.C., Bello, S.F., Abdussamad, A.M., Mark, A.I., Sanke, O.J., Onoja, A.B., Nneji, L.M., Abdullahi, N., Olaogun, S.C., Rogo, L.D., Mangbon, G.F., Pedro, S.L., Hiinan, M.P., Mukhtar, M.M., Ibrahim, J., Saidu, H., Dawuda, P.M., Bala, R.K., Abdullahi, H.L., Salako, A.E., Kdidi, S., Yahyaoui, M.H., Yin, T.T. :
Scrapie-associated polymorphisms of the prion protein gene (PRNP) in Nigerian native goats. Gene 855:147121, 2023. Pubmed reference: 36535463. DOI: 10.1016/j.gene.2022.147121.
Pakpahan, S., Widayanti, R., Artama, W.T., Budisatria, I.G.S., Lühken, G. :
Genetic variability of the prion protein gene in Indonesian goat breeds. Trop Anim Health Prod 55:87, 2023. Pubmed reference: 36806784. DOI: 10.1007/s11250-023-03486-7.
Sola, D., Betancor, M., Marco Lorente, P.A., Pérez Lázaro, S., Barrio, T., Sevilla, E., Marín, B., Moreno, B., Monzón, M., Acín, C., Bolea, R., Badiola, J.J., Otero, A. :
Diagnosis in scrapie: Conventional methods and new biomarkers. Pathogens 12:1399, 2023. Pubmed reference: 38133284. DOI: 10.3390/pathogens12121399.
Zeineldin, M., Cox-Struble, H., Camp, P., Farrell, D., Pritchard, R., Thacker, T.C., Lehman, K. :
National prevalence of caprine prion protein genetic variability at codons 146, 211, and 222 in goat herds in the United States. Vet Sci 11:13, 2023. Pubmed reference: 38250919. DOI: 10.3390/vetsci11010013.
2022 Pirisinu, L., Di Bari, M.A., D'Agostino, C., Vanni, I., Riccardi, G., Marcon, S., Vaccari, G., Chiappini, B., Benestad, S.L., Agrimi, U., Nonno, R. :
A single amino acid residue in bank vole prion protein drives permissiveness to Nor98/atypical scrapie and the emergence of multiple strain variants. PLoS Pathog 18:e1010646, 2022. Pubmed reference: 35731839. DOI: 10.1371/journal.ppat.1010646.
2021 Acín, C., Bolea, R., Monzón, M., Monleón, E., Moreno, B., Filali, H., Marín, B., Sola, D., Betancor, M., Guijarro, I.M., García, M., Vargas, A., Badiola, J.J. :
Classical and atypical scrapie in sheep and goats. Review on the etiology, genetic factors, pathogenesis, diagnosis, and control measures of both diseases. Animals (Basel) 11, 2021. Pubmed reference: 33806658. DOI: 10.3390/ani11030691.
Gallardo, M.J., Delgado, F.O. :
Animal prion diseases: A review of intraspecies transmission. Open Vet J 11:707-723, 2021. Pubmed reference: 35070868. DOI: 10.5455/OVJ.2021.v11.i4.23.
Gelasakis, A.I., Boukouvala, E., Babetsa, M., Katharopoulos, E., Palaska, V., Papakostaki, D., Giadinis, N.D., Loukovitis, D., Langeveld, J.P.M., Ekateriniadou, L.V. :
Polymorphisms of codons 110, 146, 211 and 222 at the goat PRNP locus and their association with scrapie in Greece. Animals (Basel) 11, 2021. Pubmed reference: 34438796. DOI: 10.3390/ani11082340.
Kdidi, S., Yahyaoui, M.H., Conte, M., Chiappini, B., Hammadi, M., Khorchani, T., Vaccari, G. :
Genetic variation in the prion protein gene (PRNP) of two Tunisian goat populations. Animals (Basel) 11, 2021. Pubmed reference: 34073078. DOI: 10.3390/ani11061635.
Lambert, Z.J., Greenlee, J.J., Cassmann, E.D., West Greenlee, M.H. :
Differential accumulation of misfolded prion strains in natural hosts of prion diseases. Viruses 13, 2021. Pubmed reference: 34960722. DOI: 10.3390/v13122453.
Madsen-Bouterse, S.A., Stewart, P., Williamson, H., Schneider, D.A., Goldmann, W. :
Caprine PRNP polymorphisms N146S and Q222K are associated with proteolytic cleavage of PrP^C. Genet Sel Evol 53:52, 2021. Pubmed reference: 34147084. DOI: 10.1186/s12711-021-00646-x.
Orge, L., Lima, C., Machado, C., Tavares, P., Mendonça, P., Carvalho, P., Silva, J., Pinto, M.L., Bastos, E., Pereira, J.C., Gonçalves-Anjo, N., Gama, A., Esteves, A., Alves, A., Matos, A.C., Seixas, F., Silva, F., Pires, I., Figueira, L., Vieira-Pinto, M., Sargo, R., Pires, M.D.A. :
Neuropathology of animal prion diseases. Biomolecules 11:466, 2021. Pubmed reference: 33801117. DOI: 10.3390/biom11030466.
Torricelli, M., Sebastiani, C., Ciullo, M., Ceccobelli, S., Chiappini, B., Vaccari, G., Capocefalo, A., Conte, M., Giovannini, S., Lasagna, E., Sarti, F.M., Biagetti, M. :
PRNP polymorphisms in eight local goat populations/breeds from central and southern Italy. Animals (Basel) 11:333, 2021. Pubmed reference: 33525718. DOI: 10.3390/ani11020333.
2020 Konold, T., Spiropoulos, J., Thorne, J., Phelan, L., Fothergill, L., Rajanayagam, B., Floyd, T., Vidana, B., Charnley, J., Coates, N., Simmons, M. :
The Scrapie Prevalence in a Goat Herd Is Underestimated by Using a Rapid Diagnostic Test. Front Bioeng Biotechnol 8:164, 2020. Pubmed reference: 32226784. DOI: 10.3389/fbioe.2020.00164.
Salvesen, Ø., Espenes, A., Reiten, M.R., Vuong, T.T., Malachin, G., Tran, L., Andréoletti, O., Olsaker, I., Benestad, S.L., Tranulis, M.A., Ersdal, C. :
Goats naturally devoid of PrP<sup>C</sup> are resistant to scrapie. Vet Res 51:1, 2020. Pubmed reference: 31924264. DOI: 10.1186/s13567-019-0731-2.
Teferedegn, E.Y., Yaman, Y., Ün, C. :
Novel variations in native Ethiopian goat breeds PRNP gene and their potential effect on prion protein stability. Sci Rep 10:6953, 2020. Pubmed reference: 32332800. DOI: 10.1038/s41598-020-63874-z.
2019 Kim, Y.C., Kim, S.K., Jeong, B.H. :
Scrapie susceptibility-associated indel polymorphism of shadow of prion protein gene (SPRN) in Korean native black goats. Sci Rep 9:15261, 2019. Pubmed reference: 31649311. DOI: 10.1038/s41598-019-51625-8.
Langeveld, J.P.M., Pirisinu, L., Jacobs, J.G., Mazza, M., Lantier, I., Simon, S., Andréoletti, O., Acin, C., Esposito, E., Fast, C., Groschup, M., Goldmann, W., Spiropoulos, J., Sklaviadis, T., Lantier, F., Ekateriniadou, L., Papasavva-Stylianou, P., van Keulen, L.J.M., Acutis, P.L., Agrimi, U., Bossers, A., Nonno, R. :
Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methods. Vet Res 50:97, 2019. Pubmed reference: 31767033. DOI: 10.1186/s13567-019-0718-z.
Salvesen, Ø., Tatzelt, J., Tranulis, M.A. :
The prion protein in neuroimmune crosstalk. Neurochem Int 130:104335, 2019. Pubmed reference: 30448564. DOI: 10.1016/j.neuint.2018.11.010.
2018 Cinar, M.U., Schneider, D.A., Waldron, D.F., O'Rourke, K.I., White, S.N. :
Goats singly heterozygous for PRNP S146 or K222 orally inoculated with classical scrapie at birth show no disease at ages well beyond 6 years. Vet J 233:19-24, 2018. Pubmed reference: 29486874. DOI: 10.1016/j.tvjl.2017.12.019.
Greenlee, J.J. :
Review: Update on Classical and Atypical Scrapie in Sheep and Goats. Vet Pathol :300985818794247, 2018. Pubmed reference: 30200819. DOI: 10.1177/0300985818794247.
Jeong, M.J., Kim, Y.C., Jeong, B.H. :
Prion-like protein gene (PRND) polymorphisms associated with scrapie susceptibility in Korean native black goats. PLoS One 13:e0206209, 2018. Pubmed reference: 30359416. DOI: 10.1371/journal.pone.0206209.
Sacchi, P., Rasero, R., Ru, G., Aiassa, E., Colussi, S., Ingravalle, F., Peletto, S., Perrotta, M.G., Sartore, S., Soglia, D., Acutis, P. :
Predicting the impact of selection for scrapie resistance on PRNP genotype frequencies in goats. Vet Res 49:26, 2018. Pubmed reference: 29510738. DOI: 10.1186/s13567-018-0518-x.
2017 EFSA Panel on Biological Hazards (BIOHAZ), Ricci, A., Allende, A., Bolton, D., Chemaly, M., Davies, R., Fernández Escámez, PS., Gironés, R., Herman, L., Koutsoumanis, K., Lindqvist, R., Nørrung, B., Robertson, L., Ru, G., Sanaa, M., Skandamis, P., Speybroeck, N., Simmons, M., Kuile, BT., Threlfall, J., Wahlström, H., Acutis, PL., Andreoletti, O., Goldmann, W., Langeveld, J., Windig, JJ., Ortiz Pelaez, A., Snary, E. :
Genetic resistance to transmissible spongiform encephalopathies (TSE) in goats. EFSA J 15:e04962, 2017. Pubmed reference: 32625625. DOI: 10.2903/j.efsa.2017.4962.
Meydan, H., Pehlivan, E., Özkan, M.M., Yildiz, M.A., Goldmann, W. :
Prion protein gene polymorphisms in Turkish native goat breeds. J Genet 96:299-305, 2017. Pubmed reference: 28674229.
Salvesen, Ø., Reiten, M.R., Kamstra, J.H., Bakkebø, M.K., Espenes, A., Tranulis, M.A., Ersdal, C. :
Goats without Prion Protein Display Enhanced Proinflammatory Pulmonary Signaling and Extracellular Matrix Remodeling upon Systemic Lipopolysaccharide Challenge. Front Immunol 8:1722, 2017. Pubmed reference: 29270176. DOI: 10.3389/fimmu.2017.01722.
2016 Aguilar-Calvo, P., Espinosa, J.C., Andréoletti, O., González, L., Orge, L., Juste, R., Torres, J.M. :
Goat K222-PrP(C) polymorphic variant does not provide resistance to atypical scrapie in transgenic mice. Vet Res 47:96, 2016. Pubmed reference: 27659200. DOI: 10.1186/s13567-016-0380-7.
Curcio, L., Sebastiani, C., Di Lorenzo, P., Lasagna, E., Biagetti, M. :
Review: A review on classical and atypical scrapie in caprine: Prion protein gene polymorphisms and their role in the disease. Animal 10:1585-93, 2016. Pubmed reference: 27109462. DOI: 10.1017/S1751731116000653.
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. Epidemiol Infect :1-3, 2016. Pubmed reference: 27751198. DOI: 10.1017/S0950268816002272.
Kanata, E., Arsenakis, M., Sklaviadis, T. :
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. Prion :0, 2016. Pubmed reference: 27537339. DOI: 10.1080/19336896.2016.1199312.
Konold, T., Thorne, L., Simmons, H.A., Hawkins, S.A., Simmons, M.M., González, L. :
Evidence of scrapie transmission to sheep via goat milk. BMC Vet Res 12:208, 2016. Pubmed reference: 27640200. DOI: 10.1186/s12917-016-0807-4.
Niedermeyer, S., Eiden, M., Toumazos, P., Papasavva-Stylianou, P., Ioannou, I., Sklaviadis, T., Panagiotidis, C., Langeveld, J., Bossers, A., Kuczius, T., Kaatz, M., Groschup, M.H., Fast, C. :
Genetic, histochemical and biochemical studies on goat TSE cases from Cyprus. Vet Res 47:99, 2016. Pubmed reference: 27716411. DOI: 10.1186/s13567-016-0379-0.
Windig, J.J., Hoving, R.A., Priem, J., Bossers, A., van Keulen, L.J., Langeveld, J.P. :
Variation in the prion protein sequence in Dutch goat breeds. J Anim Breed Genet 133:366-74, 2016. Pubmed reference: 26991480. DOI: 10.1111/jbg.12211.
2015 Dassanayake, R.P., White, S.N., Madsen-Bouterse, S.A., Schneider, D.A., O'Rourke, K.I. :
Role of the PRNP S127 allele in experimental infection of goats with classical caprine scrapie. Anim Genet 46:341, 2015. Pubmed reference: 25917307. DOI: 10.1111/age.12291.
Greenlee, J.J., Greenlee, M.H. :
The transmissible spongiform encephalopathies of livestock. ILAR J 56:7-25, 2015. Pubmed reference: 25991695. DOI: 10.1093/ilar/ilv008.
2014 Aguilar-Calvo, P., Espinosa, J.C., Pintado, B., Gutiérrez-Adán, A., Alamillo, E., Miranda, A., Prieto, I., Bossers, A., Andreoletti, O., Torres, J.M. :
Role of the goat K222-PrP(C) polymorphic variant in prion infection resistance. J Virol 88:2670-6, 2014. Pubmed reference: 24352451. DOI: 10.1128/JVI.02074-13.
Lacroux, C., Perrin-Chauvineau, C., Corbière, F., Aron, N., Aguilar-Calvo, P., Torres, J.M., Costes, P., Brémaud, I., Lugan, S., Schelcher, F., Barillet, F., Andréoletti, O. :
Genetic resistance to scrapie infection in experimentally challenged goats. J Virol 88:2406-13, 2014. Pubmed reference: 24284317. DOI: 10.1128/JVI.02872-13.
2013 Acín, C., Martín-Burriel, I., Monleón, E., Lyahyai, J., Pitarch, J.L., Serrano, C., Monzón, M., Zaragoza, P., Badiola, J.J. :
Prion protein gene variability in Spanish goats. Inference through susceptibility to classical scrapie strains and pathogenic distribution of peripheral PrP(sc.). PLoS One 8:e61118, 2013. Pubmed reference: 23580248. DOI: 10.1371/journal.pone.0061118.
Acquatella-Tran Van Ba, I., Imberdis, T., Perrier, V. :
From Prion Diseases to Prion-Like Propagation Mechanisms of Neurodegenerative Diseases. Int J Cell Biol 2013:975832, 2013. Pubmed reference: 24222767. DOI: 10.1155/2013/975832.
Corbière, F., Perrin-Chauvineau, C., Lacroux, C., Costes, P., Thomas, M., Brémaud, I., Martin, S., Lugan, S., Chartier, C., Schelcher, F., Barillet, F., Andreoletti, O. :
PrP-associated resistance to scrapie in five highly infected goat herds. J Gen Virol 94:241-5, 2013. Pubmed reference: 23100359. DOI: 10.1099/vir.0.047225-0.
2012 Acutis, P.L., Martucci, F., D'Angelo, A., Peletto, S., Colussi, S., Maurella, C., Porcario, C., Iulini, B., Mazza, M., Dell'atti, L., Zuccon, F., Corona, C., Martinelli, N., Casalone, C., Caramelli, M., Lombardi, G. :
Resistance to classical scrapie in experimentally challenged goats carrying mutation K222 of the prion protein gene. Vet Res 43:8, 2012. Pubmed reference: 22296670. DOI: 10.1186/1297-9716-43-8.
Benestad, S.L., Austbø, L., Tranulis, M.A., Espenes, A., Olsaker, I. :
Healthy goats naturally devoid of prion protein. Vet Res 43:87, 2012. Pubmed reference: 23249298. DOI: 10.1186/1297-9716-43-87.
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. J Gen Virol 93:1620-3, 2012. Pubmed reference: 22492914. DOI: 10.1099/vir.0.041400-0.
2011 Goldmann, W., Ryan, K., Stewart, P., Parnham, D., Xicohtencatl, R., Fernandez, N., Saunders, G., Windl, O., González, L., Bossers, A., Foster, J. :
Caprine prion gene polymorphisms are associated with decreased incidence of classical scrapie in goat herds in the United Kingdom. Vet Res 42:110, 2011. Pubmed reference: 22040234. DOI: 10.1186/1297-9716-42-110.
Papasavva-Stylianou, P., Windl, O., Saunders, G., Mavrikiou, P., Toumazos, P., Kakoyiannis, C. :
PrP gene polymorphisms in Cyprus goats and their association with resistance or susceptibility to natural scrapie. Vet J 187:245-50, 2011. Pubmed reference: 20093056. DOI: 10.1016/j.tvjl.2009.10.015.
2010 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. J Gen Virol 91:1629-34, 2010. Pubmed reference: 20107013. DOI: 10.1099/vir.0.017350-0.
González, L., Martin, S., Hawkins, S.A., Goldmann, W., Jeffrey, M., Sisó, S. :
Pathogenesis of natural goat scrapie: modulation by host PRNP genotype and effect of co-existent conditions. Vet Res 41:48, 2010. Pubmed reference: 20374697. DOI: 10.1051/vetres/2010020.
2009 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., Corbière, F., Andréoletti, 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. J Gen Virol 90:769-76, 2009. Pubmed reference: 19218225. DOI: 10.1099/vir.0.006114-0.
Vaccari, G., Panagiotidis, C.H., Acin, C., Peletto, S., Barillet, F., Acutis, P., Bossers, A., Langeveld, J., van Keulen, L., Sklaviadis, T., Badiola, J.J., Andreéoletti, O., Groschup, M.H., Agrimi, U., Foster, J., Goldmann, W. :
State-of-the-art review of goat TSE in the European Union, with special emphasis on PRNP genetics and epidemiology. Vet Res 40:48, 2009. Pubmed reference: 19505422. DOI: 10.1051/vetres/2009031.
2008 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. J Gen Virol 89:3173-3176, 2008. Pubmed reference: 19008408. DOI: 10.1099/vir.0.2008/004150-0.
2007 Papasavva-Stylianou, P., Kleanthous, M., Toumazos, P., Mavrikiou, P., Loucaides, P. :
Novel polymorphisms at codons 146 and 151 in the prion protein gene of Cyprus goats, and their association with natural scrapie. Vet J 173:459-62, 2007. Pubmed reference: 16314132. DOI: 10.1016/j.tvjl.2005.09.013.
2006 Acutis, P.L., Bossers, A., Priem, J., Riina, M.V., Peletto, S., Mazza, M., Casalone, C., Forloni, G., Ru, G., Caramelli, M. :
Identification of prion protein gene polymorphisms in goats from Italian scrapie outbreaks. J Gen Virol 87:1029-33, 2006. Pubmed reference: 16528054. DOI: 10.1099/vir.0.81440-0.
Doherr, MG. :
Brief review on the epidemiology of transmissible spongiform encephalopathies (TSE). Vaccine , 2006. Pubmed reference: 17126962. DOI: 10.1016/j.vaccine.2006.10.059.
Vaccari, G., Di Bari, MA., Morelli, L., Nonno, R., Chiappini, B., Antonucci, G., Marcon, S., Esposito, E., Fazzi, P., Palazzini, N., Troiano, P., Petrella, A., Di Guardo, G., Agrimi, U. :
Identification of an allelic variant of the goat PrP gene associated with resistance to scrapie. J Gen Virol 87:1395-402, 2006. Pubmed reference: 16603543. DOI: 10.1099/vir.0.81485-0.
2005 de Groot, SJ. :
[European Union decides to expand TSE testing in goats] Tijdschr Diergeneeskd 130:151, 2005. Pubmed reference: 15773766.
Eloit, M., Adjou, K., Coulpier, M., Fontaine, JJ., Hamel, R., Lilin, T., Messiaen, S., Andreoletti, O., Baron, T., Bencsik, A., Biacabe, AG., Beringue, V., Laude, H., Le Dur, A., Vilotte, JL., Comoy, E., Deslys, JP., Grassi, J., Simon, S., Lantier, F., Sarradin, P. :
BSE agent signatures in a goat. Vet Rec 156:523-4, 2005. Pubmed reference: 15833975.
Kurosaki, Y., Ishiguro, N., Horiuchi, M., Shinagawa, M. :
Polymorphisms of caprine PrP gene detected in Japan. J Vet Med Sci 67:321-3, 2005. Pubmed reference: 15805738.
2002 Billinis, C., Panagiotidis, C.H., Psychas, V., Argyroudis, S., Nicolaou, A., Leontides, S., Papadopoulos, O., Sklaviadis, T. :
Prion protein gene polymorphisms in natural goat scrapie Journal of General Virology 83:713-721, 2002. Pubmed reference: 11842266.
Heim, D., Maurer, E., Doherr, M.G. :
Transmissible spongiform encephalopathies in sheep and goats Schweizer Archiv fur Tierheilkunde 144:675-683, 2002. Pubmed reference: 12585208.
2001 Capucchio, M.T., Guarda, F., Pozzato, N., Coppolino, S., Caracappa, S., Di, Marco, V. :
Clinical signs and diagnosis of scrapie in Italy: A comparative study in sheep and goats Zentralblatt fur Veterinarmedizin - Reihe A 48:23-31, 2001.
Foster, J., Goldmann, W., Parnham, D., Chong, A., Hunter, N. :
Partial dissociation of PrPSc deposition and vacuolation in the brains of scrapie and BSE experimentally affected goats Journal of General Virology 82:267-273, 2001. Pubmed reference: 11125179.
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 Veterinary Record 148:165-171, 2001. Pubmed reference: 11258721.
2000 Hunter, N., Goldmann, W., Marshall, E., O'Neill, G. :
Sheep and goats: natural and experimental TSEs and factors influencing incidence of disease Archives of Virology :181-188, 2000.
1999 Agrimi, U., Ru, G., Cardone, F., Pocchiari, M., Caramelli, M. :
Epidemic of transmissible spongiform encephalopathy in sheep and goats in Italy Lancet 353:560-561, 1999. Pubmed reference: 10028993. DOI: 10.1016/S0140-6736(98)04545-0.
1998 Andrews, A.H. :
Natural occurrence of scrapie in goats in italy Veterinary Record 143:512, 1998. Pubmed reference: 9836407.
Capucchio, M.T., Guarda, F., Isaia, M.C., Caracappa, S., Dimarco, V. :
Natural occurrence of scrapie in goats in italy Veterinary Record 143:452-453, 1998. Pubmed reference: 9823611.
Goldmann, W., Chong, A., Foster, J., Hope, J., Hunter, N. :
The shortest known prion protein gene allele occurs in goats, has only three octapeptide repeats and is non-pathogenic Journal of General Virology 79:3173-3176, 1998. Pubmed reference: 9880037.
Iannuzzi, L., Palomba, R., Dimeo, G.P., Perucatti, A., Ferrara, L. :
Comparative FISH-mapping of the prion protein gene (PrnP) on cattle, river buffalo, sheep and goat chromosomes Cytogenetics & Cell Genetics 81:202-204, 1998.
1997 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 (vol 77, pg 2885, 1996) Journal of General Virology 78:697, 1997.
1996 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 Journal of General Virology 77:2885-2891, 1996. Pubmed reference: 8922485.
Schmerr, M.J., Goodwin, K.R., Cutlip, R.C., Jenny, A.L. :
Improvements in a competition assay to detect scrapie prion protein by capillary electrophoresis Journal of Chromatography B: Biomedical Applications 681:29-35, 1996.
1994 Groschup, M.H., Langeveld, J., Pfaff, E. :
The major species specific epitope in prion proteins of ruminants Archives of Virology 136:423-431, 1994. Pubmed reference: 7518226.
1993 Foster, J.D., Hope, J., Fraser, H. :
Transmission of Bovine Spongiform Encephalopathy to Sheep and Goats Veterinary Record 133:339-341, 1993. Pubmed reference: 8236676.
1992 Andrews, A.H., Laven, R., Matthews, J.G. :
Clinical Observations on 4 Cases of Scrapie in Goats Veterinary Record 130:101, 1992. Pubmed reference: 1557871.
Hunter, N., Speedy, A.W. :
Scrapie in Sheep and Goats Progress in Sheep and Goat Research :131-151, 1992.
Wood, J.L.N., Done, S.H. :
Natural Scrapie in Goats - Neuropathology Veterinary Record 131:93-96, 1992. Pubmed reference: 1523800.
Wood, J.N.L., Done, S.H., Pritchard, G.C., Wooldridge, M.J.A. :
Natural Scrapie in Goats - Case Histories and Clinical Signs Veterinary Record 131:66-68, 1992. Pubmed reference: 1529502.
1991 Perrin, G.G., Perrin, G.J., Benoit, C. :
Detection of Scrapie-Associated Fibrils in Scrapie in Goats Veterinary Record 129:432, 1991. Pubmed reference: 1776227.
1990 Cawthorne, R.J.G. :
Scrapie in Sheep and Goats Veterinary Record 127:603, 1990. Pubmed reference: 2075694.
1989 Toumazos, P., Alley, M.R. :
Scrapie in Goats in Cyprus New Zealand Veterinary Journal 37:160-162, 1989.
1964 Pattison, I.H. :
The spread of scrapie by contact between affected and healthy sheep, goats or mice Veterinary Record 76:333-336, 1964.

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