F
IPR000817

Prion protein

InterPro entry
Short namePrion
Overlapping
homologous
superfamilies
 

Description

Prion protein (PrP-c)
[2, 3, 4]
is a small glycoprotein found in high quantity in the brain of animals infected with certain degenerative neurological diseases, such as sheep scrapie and bovine spongiform encephalopathy (BSE), and the human dementias Creutzfeldt-Jacob disease (CJD) and Gerstmann-Straussler syndrome (GSS). PrP-c is encoded in the host genome and is expressed both in normal and infected cells. During infection, however, the PrP-c molecule become altered (conformationally rather than at the amino acid level) to an abnormal isoform, PrP-sc. In detergent-treated brain extracts from infected individuals, fibrils composed of polymers of PrP-sc, namely scrapie-associated fibrils or prion rods, can be evidenced by electron microscopy. The precise function of the normal PrP isoform in healthy individuals remains unknown. Several results, mainly obtained in transgenic animals, indicate that PrP-c might play a role in long-term potentiation, in sleep physiology, in oxidative burst compensation (PrP can fix four Cu2+ through its octarepeat domain), in interactions with the extracellular matrix (PrP-c can bind to the precursor of the laminin receptor, LRP), in apoptosis and in signal transduction (costimulation of PrP-c induces a modulation of Fyn kinase phosphorylation)
[1]
.

The normal isoform, PrP-c, is anchored at the cell membrane, in rafts, through a glycosyl phosphatidyl inositol (GPI); its half-life at the cell surface is 5 h, after which the protein is internalised through a caveolae-dependent mechanism and degraded in the endolysosome compartment. Conversion between PrP-c and PrP-sc occurs likely during the internalisation process.

References

1.Prion diseases: pathogenesis and public health concerns. Dormont D. FEBS Lett. 529, 17-21, (2002). View articlePMID: 12354606

2.Scrapie prions. Prusiner SB. Annu. Rev. Microbiol. 43, 345-74, (1989). View articlePMID: 2572197

3.Prions and prion proteins. Stahl N, Prusiner SB. FASEB J. 5, 2799-807, (1991). View articlePMID: 1916104

4.The scrapie agent and the prion hypothesis. Brunori M, Silvestrini MC, Pocchiari M. Trends Biochem. Sci. 13, 309-13, (1988). View articlePMID: 2908696

GO terms

molecular function

  • None

cellular component

Cross References

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