OMIA:000944-9031 : Spongiform encephalopathy, susceptibility/resistance to in Gallus gallus (chicken)

In other species: Mallard , 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 , 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

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.

Associated gene:

Symbol Description Species Chr Location OMIA gene details page Other Links
Prnp prion protein Rattus norvegicus 3 NC_086021.1 (139639076..139654420) Prnp Homologene, Ensembl , NCBI gene

Cite this entry

Nicholas, F. W., Tammen, I., & Sydney Informatics Hub. (2023). OMIA:000944-9031: 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.

2022 Kim, Y.C., Kim, H.H., Jeong, B.H. :
The first report of polymorphisms and genetic characteristics of the shadow of prion protein (SPRN) in prion disease-resistant animal, chickens. Front Vet Sci 9:904305, 2022. Pubmed reference: 35782543. DOI: 10.3389/fvets.2022.904305.
2021 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.
2019 Kim, Y.C., Won, S.Y., Jeong, B.H. :
Absence of single nucleotide polymorphisms (SNPs) in the open reading frame (ORF) of the prion protein gene (PRNP) in a large sampling of various chicken breeds. BMC Genomics 20:922, 2019. Pubmed reference: 31795947. DOI: 10.1186/s12864-019-6315-8.
2018 Kim, Y.C., Jeong, M.J., Jeong, B.H. :
The first report of genetic variations in the chicken prion protein gene. Prion 12:197-203, 2018. Pubmed reference: 29966485. DOI: 10.1080/19336896.2018.1471922.
2003 Matthews, D., Cooke, B.C. :
The potential for transmissible spongiform encephalopathies in non-ruminant livestock and fish Revue Scientifique et Technique 22:283-96, 2003. Pubmed reference: 12793786.
1999 Marcotte, E.M., Eisenberg, D. :
Chicken prion tandem repeats form a stable, protease-resistant domain Biochemistry 38:667-676, 1999. Pubmed reference: 9888807. DOI: 10.1021/bi981487f.
Wopfner, F., Weidenhofer, G., Schneider, R., von, Brunn, A., Gilch, S., Schwarz, T.F., Werner, T., Schatzl, M. :
Analysis of 27 mammalian and 9 avian PrPs reveals high conservation of flexible regions of the prion protein Journal of Molecular Biology 289:1163-1178, 1999. Pubmed reference: 10373359. DOI: 10.1006/jmbi.1999.2831.
1997 Cawthorne, R.J.G. :
Failure to confirm a TSE in chickens Veterinary Record 141:203, 1997. Pubmed reference: 9292978.
Narang, H. :
Failure to confirm a TSE in chickens Veterinary Record 141:255-256, 1997. Pubmed reference: 9308154.
1996 Pearce, F. :
BSE may lurk in pigs and chickens New Scientist 150:5, 1996.

Edit History

  • Created by Frank Nicholas on 06 Sep 2005
  • Changed by Frank Nicholas on 15 May 2020
  • Changed by Imke Tammen2 on 08 Aug 2021
  • Changed by Imke Tammen2 on 25 Aug 2021
  • Changed by Imke Tammen2 on 28 Aug 2021
  • Changed by Imke Tammen2 on 17 Jan 2023
  • Changed by Imke Tammen2 on 16 Oct 2023