The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
Gene Review

PRNP  -  prion protein

Ovis aries

Synonyms: PRPC, Prp, SIP
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of PRNP


Psychiatry related information on PRNP

  • These data imply that homozygosity for Q-171 codons is necessary but not sufficient for the development of natural scrapie, echo reports of recessive manifestation, and parallel over-representation of PRNP codon 129 homozygotes in Creutzfeldt-Jakob disease of humans [5].

High impact information on PRNP

  • Similar genetic differences exist in sheep, in which PRNP polymorphisms modify the susceptibility to scrapie [6].
  • In intestinal lymph nodes, expression levels differed between the different PRNP genotypes [6].
  • However, the known coding polymorphisms within the bovine PRNP gene have little or no effect on bovine spongiform encephalopathy (BSE) susceptibility in cattle [6].
  • In prion diseases the lethal evolution is associated with formation of PrP(Sc), a misfolded and amyloid form of the normal cellular prion protein PrP [7].
  • Detailed RNA-transcription and protein-expression analysis of one PRNP+/- goat showed that one allele of the caprine PRNP gene had been disrupted functionally [8].

Biological context of PRNP


Anatomical context of PRNP

  • Haplotypes of the 3 PRNP codons were determined for each sheep, and breed of origin of each gamete was predicted by genotyping 6 microsatellite markers flanking the PRNP locus [10].
  • One possibility for the lack of PrP(Sc) accumulation in brains and lymph nodes of scrapie-exposed sheep with 171RR PRNP genetics is because of fewer PrPc-positive myeloid-derived cells available for conversion of PrPc to PrP(Sc) [11].
  • Using flow cytometric analysis, statistically fewer PrPc-positive microglia and monocytes were observed from sheep with 171RR PRNP genetics than from sheep with 171QQ PRNP genetics (P<0.05) [11].
  • Further search of PrP(C)-interacting molecules and detailed studies using Prnp(-/-) mice and various type of Prnp(-/-) cell lines under various conditions are the prerequisites in elucidating PrP functions [12].

Associations of PRNP with chemical compounds


Regulatory relationships of PRNP

  • Polymorphisms of the prion protein gene PRNP have been shown to influence the susceptibility/resistance to prion infections in human and sheep [15].

Analytical, diagnostic and therapeutic context of PRNP

  • METHODS: A dual fluorescent multiprobe assay consisting of 2 closed tube PCR reactions containing respectively 4 and 3 dual-labelled fluorescent ASO probes for the detection in real-time of the 7 allelic variants of sheep PRNP mentioned above [16].
  • Here, five live PRNP+/- goats cloned by gene targeting are reported [8].


  1. Prion protein gene (PRNP) polymorphisms in Xinjiang local sheep breeds in China. Lan, Z., Wang, Z.L., Liu, Y., Zhang, X. Arch. Virol. (2006) [Pubmed]
  2. Bovine spongiform encephalopathy agent in spleen from an ARR/ARR orally exposed sheep. Andréoletti, O., Morel, N., Lacroux, C., Rouillon, V., Barc, C., Tabouret, G., Sarradin, P., Berthon, P., Bernardet, P., Mathey, J., Lugan, S., Costes, P., Corbière, F., Espinosa, J.C., Torres, J.M., Grassi, J., Schelcher, F., Lantier, F. J. Gen. Virol. (2006) [Pubmed]
  3. IL-1 family members as candidate genes modulating scrapie susceptibility in sheep: localization, partial characterization, and expression. Marcos-Carcavilla, A., Calvo, J.H., González, C., Moazami-Goudarzi, K., Laurent, P., Bertaud, M., Hayes, H., Beattie, A.E., Serrano, C., Lyahyai, J., Martín-Burriel, I., Alves, E., Zaragoza, P., Badiola, J.J., Serrano, M. Mamm. Genome (2007) [Pubmed]
  4. Polymorphisms of the prion protein gene (PRNP) in Hanwoo (Bos taurus coreanae) and Holstein cattle. Jeong, B.H., Sohn, H.J., Lee, J.O., Kim, N.H., Kim, J.I., Lee, S.Y., Cho, I.S., Joo, Y.S., Carp, R.I., Kim, Y.S. Genes Genet. Syst. (2005) [Pubmed]
  5. Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie. Westaway, D., Zuliani, V., Cooper, C.M., Da Costa, M., Neuman, S., Jenny, A.L., Detwiler, L., Prusiner, S.B. Genes Dev. (1994) [Pubmed]
  6. Bovine prion protein gene (PRNP) promoter polymorphisms modulate PRNP expression and may be responsible for differences in bovine spongiform encephalopathy susceptibility. Sander, P., Hamann, H., Drögemüller, C., Kashkevich, K., Schiebel, K., Leeb, T. J. Biol. Chem. (2005) [Pubmed]
  7. Sequential generation of two structurally distinct ovine prion protein soluble oligomers displaying different biochemical reactivities. Rezaei, H., Eghiaian, F., Perez, J., Doublet, B., Choiset, Y., Haertle, T., Grosclaude, J. J. Mol. Biol. (2005) [Pubmed]
  8. Functional disruption of the prion protein gene in cloned goats. Yu, G., Chen, J., Yu, H., Liu, S., Chen, J., Xu, X., Sha, H., Zhang, X., Wu, G., Xu, S., Cheng, G. J. Gen. Virol. (2006) [Pubmed]
  9. Linkage disequilibrium across six prion gene regions spanning 20 kbp in U.S. sheep. Green, B.T., Heaton, M.P., Clawson, M.L., Laegreid, W.W. Mamm. Genome (2006) [Pubmed]
  10. Evaluation of associations between prion haplotypes and growth, carcass, and meat quality traits in a Dorset x Romanov sheep population. Isler, B.J., Freking, B.A., Thallman, R.M., Heaton, M.P., Leymaster, K.A. J. Anim. Sci. (2006) [Pubmed]
  11. Fewer PrPc myeloid-based cells in sheep with the prion-resistant genotype. Herrmann, L.M., Baszler, T.V., O'Rourke, K.I., Suarez, C.E., Bakko, M., Alverson, J., Knowles, D.P. Neuroreport (2006) [Pubmed]
  12. Bovine spongiform encephalopathy in Japan: history and recent studies on oxidative stress in prion diseases. Onodera, T., Sakudo, A., Wu, G., Saeki, K. Microbiol. Immunol. (2006) [Pubmed]
  13. The effect of the osmolyte trimethylamine N-oxide on the stability of the prion protein at low pH. Granata, V., Palladino, P., Tizzano, B., Negro, A., Berisio, R., Zagari, A. Biopolymers (2006) [Pubmed]
  14. Single nucleotide polymorphisms of the prion protein gene (PRNP) in Chinese pig breeds. Meng, L., Zhao, D., Liu, H., Yang, J., Ning, Z. Xenotransplantation (2005) [Pubmed]
  15. DNA polymorphisms of the prion doppel gene region in four different German cattle breeds and cows tested positive for bovine spongiform encephalopathy. Balbus, N., Humeny, A., Kashkevich, K., Henz, I., Fischer, C., Becker, C.M., Schiebel, K. Mamm. Genome (2005) [Pubmed]
  16. A dual fluorescent multiprobe assay for prion protein genotyping in sheep. Van Poucke, M., Vandesompele, J., Mattheeuws, M., Van Zeveren, A., Peelman, L.J. BMC Infect. Dis. (2005) [Pubmed]
WikiGenes - Universities