Encyclopedia of Signaling Molecules

2012 Edition
| Editors: Sangdun Choi

Prion Protein (PRNP)

Reference work entry
DOI: https://doi.org/10.1007/978-1-4419-0461-4_390

Synonyms

 CD230;  Prion protein;  Prn-i;  Prnp;  Prn-p;  PrP;  PrPC;  PrPSc;  Sinc

Historical Background

Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases characterized by neuron loss, glial reactions, and tissue spongiosis, which course with motor and/or cognitive symptoms (Knight and Will 2004). The TSEs are associated with conformational conversion of the prion protein (PrPC, the product of the Prnp gene), wherein the predominantly α-helical secondary structure of PrPC changes into an aggregation-prone, β-sheet richer structure known as PrPSc. The latter is believed to coerce PrPC molecules into conformational conversion, thus behaving as a proteinaceous infectious particle, or prion (Prusiner 1998), which gave TSEs the epithet prion diseases.

The much needed development of effective treatment for these still incurable diseases depends on the understanding of functional properties of the prion protein (Soto and Satani 2010). Most studies of...

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Notes

Acknowledgments

The authors’ research groups have been supported by the Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq), Fundação de Amparo à Pesquisa do Estado do Rio de Janeiro (FAPERJ), Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP), Programa Institutos Nacionais de Ciência e Tecnologia (CNPq/MCT), Ludwig Institute for Cancer Research, and PrioNet-Canada. V.R.M. is an International Scholar of the Howard Hughes Medical Institute.

References

  1. Bremer J, Baumann F, Tiberi C, Wessig C, Fischer H, Schwarz P, Steele AD, Toyka KV, Nave KA, Weis J, Aguzzi A. Axonal prion protein is required for peripheral myelin maintenance. Nat Neurosci. 2010;13(3):310–318.PubMedGoogle Scholar
  2. Isaacs JD, Jackson GS, Altmann DM. The role of the cellular prion protein in the immune system. Clin Exp Immunol. 2006;146(1):1–8.PubMedGoogle Scholar
  3. Knight RS, Will RG. Prion diseases. J Neurol Neurosurg Psychiatry. 2004;75(Suppl 1):i36–i42.PubMedGoogle Scholar
  4. Linden R, Martins VR, Prado MA, Cammarota M, Izquierdo I, Brentani RR. Physiology of the prion protein. Physiol Rev. 2008;88(2):673–728.PubMedGoogle Scholar
  5. Martins VR, Beraldo FH, Hajj GN, Lopes MH, Lee KS, Prado MM, et al. Prion protein: orchestrating neurotrophic activities. Curr Issues Mol Biol. 2010;12(2):63–86.PubMedGoogle Scholar
  6. Mastrangelo P, Westaway D. Biology of the prion gene complex. Biochem Cell Biol. 2001;79(5):613–628.PubMedGoogle Scholar
  7. Mehrpour M, Codogno P. Prion protein: from physiology to cancer biology. Cancer Lett. 2010;290(1):1–23.PubMedGoogle Scholar
  8. Nitta K, Sakudo A, Masuyama J, Xue G, Sugiura K, Onodera T. Role of cellular prion proteins in the function of macrophages and dendritic cells. Protein Pept Lett. 2009;16(3):239–246.PubMedGoogle Scholar
  9. Parkyn CJ, Vermeulen EG, Mootoosamy RC, Sunyach C, Jacobsen C, Oxvig C, et al. LRP1 controls biosynthetic and endocytic trafficking of neuronal prion protein. J Cell Sci. 2008;121(Pt 6):773–783.PubMedGoogle Scholar
  10. Porto-Carreiro I, Fevrier B, Paquet S, Vilette D, Raposo G. Prions and exosomes: from PrPc trafficking to PrPsc propagation. Blood Cells Mol Dis. 2005;35(2):143–148.PubMedGoogle Scholar
  11. Prado MA, Alves-Silva J, Magalhaes AC, Prado VF, Linden R, Martins VR, et al. PrPc on the road: trafficking of the cellular prion protein. J Neurochem. 2004;88(4):769–781.PubMedGoogle Scholar
  12. Prusiner SB. Prions. Proc Natl Acad Sci USA. 1998;95(23):13363–13383.PubMedGoogle Scholar
  13. Rudd PM, Wormald MR, Wing DR, Prusiner SB, Dwek RA. Prion glycoprotein: structure, dynamics, and roles for the sugars. Biochemistry. 2001;40(13):3759–3766.PubMedGoogle Scholar
  14. Rutishauser D, Mertz KD, Moos R, Brunner E, Rulicke T, Calella AM, et al. The comprehensive native interactome of a fully functional tagged prion protein. PLoS One. 2009;4(2):e4446.PubMedGoogle Scholar
  15. Soto C, Satani N. The intricate mechanisms of neurodegeneration in prion diseases. Trends Mol Med. 2010;17(1):14–24.Google Scholar
  16. Steinacker P, Hawlik A, Lehnert S, Jahn O, Meier S, Gorz E, et al. Neuroprotective function of cellular prion protein in a mouse model of amyotrophic lateral sclerosi. Am J Pathol. 2010;176(3):1409–1420.PubMedGoogle Scholar
  17. Stella R, Massimino ML, Sandri M, Sorgato MC, Bertoli A. Cellular prion protein promotes regeneration of adult muscle tissue. Mol Cell Biol. 2010;30(20):4864–4876.PubMedGoogle Scholar
  18. Taylor DR, Hooper NM. The low-density lipoprotein receptor-related protein 1 (LRP1) mediates the endocytosis of the cellular prion protein. Biochem J. 2007;402(1):17–23.PubMedGoogle Scholar
  19. Wadsworth JD, Asante EA, Collinge J. Contribution of transgenic models to understanding human prion disease. Neuropathol Appl Neurobiol. 2010;36(7):576–597.PubMedGoogle Scholar
  20. Weissmann C, Fischer M, Raeber A, Bueler H, Sailer A, Shmerling D, et al. The use of transgenic mice in the investigation of transmissible spongiform encephalopathies. Rev Sci Tech. 1998;17(1):278–290.PubMedGoogle Scholar
  21. Wuthrich K, Riek R. Three-dimensional structures of prion proteins. Adv Protein Chem. 2001;57:55–82.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Laboratorio de Neurogenese, Instituto de Biofisica da UFRJUniversidade Federal do Rio de JaneiroRio de JaneiroBrazil
  2. 2.International Center for Research and Education Antonio Prudente FoundationA.C. Camargo HospitalSão PauloBrazil
  3. 3.Robarts Research InstituteUniversity of Western OntarioLondonCanada