OMIA:000944-9685 : Spongiform encephalopathy, susceptibility/resistance to in Felis catus (domestic cat)

In other species: Mallard , chicken , Ring-necked pheasant , white-tufted-ear marmoset , macaques , crab-eating macaque , Rhesus monkey , dog , domestic ferret , 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.

Cite this entry

Nicholas, F. W., Tammen, I., & Sydney Informatics Hub. (2023). OMIA:000944-9685: 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 Güvendi, M., Can, H., Köseoğlu, A.E., Alak, S.E., Ün, C. :
First report of a novel 108 bp deletion and five novel SNPs in PRNP gene of stray cats and in silico analysis of their possible relation with feline spongiform encephalopathy. Top Companion Anim Med 59:100859, 2024. Pubmed reference: 38508487. DOI: 10.1016/j.tcam.2024.100859.
2022 Kim, Y.C., Kim, H.H., Kim, K., Kim, A.D., Jeong, B.H. :
Novel polymorphisms and genetic characteristics of the shadow of prion protein gene (SPRN) in cats, hosts of feline spongiform encephalopathy. Viruses 14:981, 2022. Pubmed reference: 35632724. DOI: 10.3390/v14050981.
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.
2020 Kim, H.H., Kim, Y.C., Kim, K., Kim, A.D., Jeong, B.H. :
Novel polymorphisms and genetic features of the prion protein gene (PRNP) in cats, hosts of feline spongiform encephalopathy. Genes (Basel) 12:13, 2020. Pubmed reference: 33374431. DOI: 10.3390/genes12010013.
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.
2012 Stewart, P., Campbell, L., Skogtvedt, S., Griffin, K.A., Arnemo, J.M., Tryland, M., Girling, S., Miller, M.W., Tranulis, M.A., Goldmann, W. :
Genetic predictions of prion disease susceptibility in carnivore species based on variability of the prion gene coding region. PLoS One 7:e50623, 2012. Pubmed reference: 23236380. DOI: 10.1371/journal.pone.0050623.
2005 Kelly, DF., Wells, GA., Haritani, M., Higgins, RJ., Jeffrey, M. :
Neuropathological findings in cats with clinically suspect but histologically unconfirmed feline spongiform encephalopathy. Vet Rec 156:472-7, 2005. Pubmed reference: 15828743.
2002 Demierre, S., Botteron, C., Cizinauskas, S., Doherr, M.G., Fatzer, R., Jaggy, A. :
Feline spongiform encephalopathy: first clinical case in Switzerland Schweizer Archiv fur Tierheilkunde 144:550-557, 2002. Pubmed reference: 12442705.
Heim, D., Geiser, F., Perler, L., Wyss, R. :
Beyond BSE: Transmissible spongiform encephalopathies in other species Schweizer Archiv fur Tierheilkunde 144:664-673, 2002. Pubmed reference: 12585207.
2001 Ryder, S.J., Wells, G.A.H., Bradshaw, J.M., Pearson, G.R. :
Inconsistent detection of PrP in extraneural tissues of cats with feline spongiform encephalopathy Veterinary Record 148:437-441, 2001. Pubmed reference: 11338713.
1998 Zanusso, G., Nardelli, E., Rosati, A., Fabrizi, G.M., Ferrari, S., Carteri, A., Desimone, F., Rizutto, N., Monaco, S. :
Simultaneous occurrence of spongiform encephalopathy in a man and his cat in italy Lancet 352:1116-1117, 1998. Pubmed reference: 9798590. DOI: 10.1016/S0140-6736(05)79756-7.
1996 Anon. :
Feline spongiform encephalopathy Journal of Small Animal Practice 37:198-199, 1996. Pubmed reference: 8731410.
Collinge, J., Sidle, K.C.L., Meads, J., Ironside, J., Hill, A.F. :
Molecular analysis of prion strain variation and the aetiology of new variant CJD Nature 383:685-690, 1996. Pubmed reference: 8878476. DOI: 10.1038/383685a0.
Glavits, R., Ratz, F. :
Studies on the spongiform encephalopathies of cattle, sheep and cats (BSE, scrapie, FSE) in the Hungarian diagnostic institutes [Hungarian] Magyar Allatorvosok Lapja 51:529-531, 1996.
1995 Bratberg, B., Ueland, K., Wells, G.A.H. :
Feline spongiform encephalopathy in a cat in Norway Veterinary Record 136:444, 1995. Pubmed reference: 7631481.
1994 Fraser, H., Pearson, G.R., McConnell, I., Bruce, M.E., Wyatt, J.M., Gruffydd-Jones, T.J. :
Transmission of Feline Spongiform Encephalopathy to Mice Veterinary Record 134:449, 1994. Pubmed reference: 8048218.
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.
Hewicker Trautwein, M. :
Feline Spongiform Encephalopathy Kleintierpraxis 39:855, 1994.
1993 Wyatt, J.M., Pearson, G.R., Gruffydd-Jones, T.J. :
Feline Spongiform Encephalopathy Feline Practice 21:7-9, 1993.
1992 Gruffydd-Jones, T.J., Galloway, P.E., Pearson, G.R. :
Feline Spongiform Encephalopathy Journal of Small Animal Practice 33:471-476, 1992.
Moennig, V. :
Mad Cow Disease in Cats Tijdschrift Voor Diergeneeskunde 117:412-413, 1992. Pubmed reference: 1641835.
Pearson, G.R., Wyatt, J.M., Gruffydd-Jones, T.J., Hope, J., Chong, A., Higgins, R.J., Scott, A.C., Wells, G.A.H. :
Feline Spongiform Encephalopathy - Fibril and PrP Studies Veterinary Record 131:307-310, 1992. Pubmed reference: 1279883.
Wells, G.A.H., Mcgill, I.S. :
Recently Described Scrapie-Like Encephalopathies of Animals - Case Definitions - Review Article Research in Veterinary Science 53:1-10, 1992. Pubmed reference: 1410804.
1991 Pearson, G.R., Gruffydd-Jones, T.J., Wyatt, J.M., Hope, J., Chong, A., Scott, A.C., Dawson, M., Wells, G.A.H. :
Feline Spongiform Encephalopathy Veterinary Record 128:532, 1991. Pubmed reference: 1866888.
Synge, B.A., Waters, J.W. :
Spongiform Encephalopathy in a Scottish Cat Veterinary Record 129:320, 1991. Pubmed reference: 1801815.
Wyatt, J.M. :
Naturally Occurring Spongiform Encephalopathy in Cats in the UK Australian Veterinary Practitioner 21:174-176, 1991.
Wyatt, J.M., Pearson, G.R., Smerdon, T.N., Gruffydd-Jones, T.J., Wells, G.A.H., Wilesmith, J.W. :
Naturally Occurring Scrapie-Like Spongiform Encephalopathy in 5 Domestic Cats Veterinary Record 129:233-236, 1991. Pubmed reference: 1957458.
1990 Aldhous, P. :
BSE - Spongiform Encephalopathy Found in Cat Nature 345:194, 1990. Pubmed reference: 2333092. DOI: 10.1038/345194c0.
Leggett, M.M., Dukes, J., Pirie, H.M. :
A Spongiform Encephalopathy in a Cat Veterinary Record 127:586-588, 1990. Pubmed reference: 2075687.
Meldrum, K.C. :
Spongiform encephalopathy in a cat Vet Rec 126:513, 1990. Pubmed reference: 2368282.
Parrysmith, P.W., Isserlin, J.A.M., Auerbach, M., Bellois, E., Loosevelt, R. :
Spongiform Encephalopathy in a Cat Veterinary Record 126:513, 1990.
Wyatt, J.M., Pearson, G.R., Smerdon, T., Gruffydd-Jones, T.J., Wells, G.A.H. :
Spongiform Encephalopathy in a Cat Veterinary Record 126:513, 1990.

Edit History

  • Created by Frank Nicholas on 06 Sep 2005
  • Changed by Frank Nicholas on 15 May 2020
  • Changed by Imke Tammen2 on 16 Oct 2023