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Prion (PRNP)

  • Living reference work entry
  • First Online:
Encyclopedia of Signaling Molecules

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Synonyms

CD230; Prion protein; Prn-i; Prnp; Prn-p; PrP; PrpC ; 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. 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-rich structure known as PrPSc. The latter is believed to coerce PrPC molecules into conformational conversion, thus behaving as a proteinaceous infectious particle, or prion, which gave TSEs the epithet prion diseases (Colby and Prusiner 2011).

The much needed development of effective treatment for these still incurable diseases depends on the understanding of functional properties of the prion protein. Most studies of physiological functions of PrPChave been...

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References

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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), Canadian Institutes of Health Research (CIHR), the Weston Brain Institute, Brain Canada, The Alzheimer’s Association (USA), The Alzheimer’s Society (Canada), and PrioNet-Canada. V.R.M. is an International Scholar of the Howard Hughes Medical Institute.

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Authors and Affiliations

  1. Laboratorio de Neurogenese, Instituto de Biofisica da UFRJ, Rio de Janeiro, RJ, 21949-900, Brazil

    Rafael Linden

  2. International Research Center, A.C. Camargo Cancer Center, São Paulo, SP, 01508-010, Brazil

    Vilma R. Martins

  3. Department of Physiology and Pharmacology and Anatomy & Cell Biology, Schulich School of Medicine, University of Western Ontario, London, ON, N6A 5K8, Canada

    Marco A. M. Prado

Authors
  1. Rafael Linden
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  2. Vilma R. Martins
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  3. Marco A. M. Prado
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Corresponding author

Correspondence to Rafael Linden .

Editor information

Editors and Affiliations

  1. Dept. Biological Science, Department of Molecular Science and Tech College of Natural Science, Suwon, Korea (Republic of)

    Sangdun Choi

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Linden, R., Martins, V.R., Prado, M.A.M. (2016). Prion (PRNP). In: Choi, S. (eds) Encyclopedia of Signaling Molecules. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6438-9_390-1

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  • DOI: https://doi.org/10.1007/978-1-4614-6438-9_390-1

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  • Online ISBN: 978-1-4614-6438-9

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