OMIA 000944-452646 : Spongiform encephalopathy in Neovison vison

In other species: domestic cat , cattle , goat , pig , sheep , golden hamster , blue antelope , white-tufted-ear marmoset , eland , domestic ferret , greater kudu , Arabian oryx , , puma , Eastern wapiti , cheetah , chicken , crab-eating macaque , Rhesus monkey , macaques , black-tailed deer , , rabbit , dog , water buffalo , , Manchurian Wapiti , deer , domestic guinea pig , , Western roe deer , fallow deer , , , horse

Possibly relevant human trait(s) and/or gene(s) (MIM number): 176640

Mendelian trait/disorder: unknown

Considered a defect: yes

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).

Species-specific name: Scrapie


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.
2015 Aguilar-Calvo, P., GarcĂ­a, 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