OMIA:000944-452646 : Spongiform encephalopathy, susceptibility/resistance to in Neovison vison (American mink)

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 , goat , 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

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: unknown

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

Cite this entry

Nicholas, F. W., Tammen, I., & Sydney Informatics Hub. (2020). OMIA:000944-452646: 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.

2021 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.
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.
2018 Houston, F., Andréoletti, O. :
Animal prion diseases: the risks to human health. Brain Pathol 29:248-262, 2018. Pubmed reference: 30588682. DOI: 10.1111/bpa.12696.
2015 Aguilar-Calvo, P., García, C., Espinosa, J.C., Andreoletti, O., Torres, J.M. :
Prion and prion-like diseases in animals. Virus Res 207:82-93, 2015. Pubmed reference: 25444937. DOI: 10.1016/j.virusres.2014.11.026.
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.
2009 Liberski, PP., Sikorska, B., Guiroy, D., Bessen, RA. :
Transmissible mink encephalopathy - review of the etiology of a rare prion disease. Folia Neuropathol 47:195-204, 2009. Pubmed reference: 19618341.
2005 Windl, O., Buchholz, M., Neubauer, A., Schulz-Schaeffer, W., Groschup, M., Walter, S., Arendt, S., Neumann, M., Voss, AK., Kretzschmar, HA. :
Breaking an absolute species barrier: transgenic mice expressing the mink PrP gene are susceptible to transmissible mink encephalopathy. J Virol 79:14971-5, 2005. Pubmed reference: 16282497. DOI: 10.1128/JVI.79.23.14971-14975.2005.
1996 Mckenzie, D., Bartz, J.C., Marsh, R.F. :
Transmissible mink encephalopathy Seminars in Virology 7:201-206, 1996.
1995 Darcel, C. :
Reflections on scrapie and related disorders, with consideration of the possibility of a viral aetiology - Discussion paper Veterinary Research Communications 19:231-252, 1995. Pubmed reference: 7571397.
Robinson, M.M., Hadlow, W.J., Knowles, D.P., Huff, T.P., Lacy, P.A., Marsh, R.F., Gorham, J.R. :
Experimental infection of cattle with the agents of transmissible mink encephalopathy and scrapie Journal of Comparative Pathology 113:241-251, 1995. Pubmed reference: 8592050.
1994 Bartz, J.C., Mckenzie, D.I., Bessen, R.A., Marsh, R.F., Aiken, J.M. :
Transmissible mink encephalopathy species barrier effect between ferret and mink: PrP gene and protein analysis Journal of General Virology 75:2947-2953, 1994. Pubmed reference: 7964604.
Bessen, R.A., Marsh, R.F. :
Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy Journal of Virology 68:7859-7868, 1994. Pubmed reference: 7966576.
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.
Robinson, M.M., Hadlow, W.J., Huff, T.P., Wells, G.A.H., Dawson, M., Marsh, R.F., Gorham, J.R. :
Experimental infection of mink with bovine spongiform encephalopathy Journal of General Virology 75:2151-2155, 1994. Pubmed reference: 8077914.

Edit History

  • Created by Frank Nicholas on 06 Sep 2005
  • Changed by Frank Nicholas on 09 Nov 2013
  • Changed by Frank Nicholas on 15 May 2020