OMIA 000944-9946 : Spongiform encephalopathy in Tragelaphus strepsiceros
In other species: domestic cat , cattle , goat , pig , sheep , American mink , golden hamster , blue antelope , white-tufted-ear marmoset , eland , domestic ferret , 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 , , 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).
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.
|1994||Kirkwood, J.K., Cunningham, A.A., Austin, A.R., Wells, G.A.H., Sainsbury, A.W. :|
|Spongiform Encephalopathy in a Greater Kudu (Tragelaphus Strepsiceros) Introduced into an Affected Group Veterinary Record 134:167-168, 1994. Pubmed reference: 8160331.|
|1993||Cunningham, A.A., Wells, G.A.H., Scott, A.C., Kirkwood, J.K., Barnett, J.E.F. :|
|Transmissible Spongiform Encephalopathy in Greater Kudu (Tragelaphus strepsiceros) Veterinary Record 132:68, 1993. Pubmed reference: 8430487.|
|Kirkwood, J.K., Cunningham, A.A., Wells, G.A.H., Wilesmith, J.W., Barnett, J.E.F. :|
|Spongiform Encephalopathy in a Herd of Greater Kudu (Tragelaphus strepsiceros) - Epidemiological Observations Veterinary Record 133:360-364, 1993. Pubmed reference: 8256421.|
|Poidinger, M., Kirkwood, J., Almond, J.W. :|
|Sequence analysis of the PrP protein from two species of antelope susceptible to transmissible spongiform encephalopathy Archives of Virology 131:193-199, 1993. Pubmed reference: 8328913.|
|1992||Kirkwood, J.K., Wells, G.A.H., Cunningham, A.A., Jackson, S.I., Scott, A.C., Dawson, M., Wilesmith, J.W. :|
|Scrapie-Like Encephalopathy in a Greater Kudu (Tragelaphus strepsiceros) Which Had Not Been Fed Ruminant-Derived Protein Veterinary Record 130:365-367, 1992. Pubmed reference: 1604783.|
|1990||Kirkwood, J.K., Wells, G.A.H., Wilesmith, J.W., Cunningham, A.A., Jackson, S.I. :|
|Spongiform Encephalopathy in an Arabian Oryx (Oryx-Leucoryx) and a Greater Kudu (Tragelaphus-Strepsiceros) Veterinary Record 127:418-420, 1990. Pubmed reference: 2264242.|
- Created by Frank Nicholas on 06 Sep 2005