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Current Issues in Molecular Biology is published by MDPI from Volume 43 Issue 1 (2021). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with Caister Press.
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Review

Prion Protein: Orchestrating Neurotrophic Activities

by
Vilma R. Martins
1,*,†,
Flavio H. Beraldo
1,†,
Glaucia N. Hajj
1,†,
Marilene H. Lopes
1,†,
Kil Sun Lee
2,
Marco A. Prado
3,4 and
Rafael Linden
5
1
Ludwig Institute for Cancer Research, Hospital Alemao, Oswaldo Cruz, Sao Paulo, SP, Brazil
2
Associação Fundo de Incentivo à Psicofarmacologia, São Paulo, SP, Brazil
3
Departments of Anatomy and Cell Biology/Physicology and Pharmacology, Robarts Research Institute, University of Western Ontario, London, ON, Canada
4
Department of Pharmacology, Federal University of Minas Gerais, Belo Horizonte, MG, Brazil
5
Instituto de Biofísica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil
*
Author to whom correspondence should be addressed.
†
These authors contributed equally to this work.
Curr. Issues Mol. Biol. 2010, 12(2), 63-86; https://doi.org/10.21775/cimb.012.063
Submission received: 3 May 2009 / Revised: 7 May 2009 / Accepted: 7 July 2009 / Published: 18 September 2009

Abstract

PrPC is highly expressed in both the central and peripheral nervous systems from early stages of development and in adulthood. Its major conformational change and conversion into an abnormal form (PrPSc) has been associated with the generation of prions, the infectious agent of transmissible spongiform encephalopathies (TSEs). The massive neurodegeneration presented by individuals suffering from these diseases has been associated with the gain of neurotoxic activity of PrPSc. On the other hand, major neurodegeneration is also observed in transgenic mice expressing PrPC molecules deleted of specific domains, which points to important functional domains within this molecule, and supports the hypothesis that loss-of PrPC function may contribute to the pathogenesis of TSEs. Furthermore, a large body of data demonstrates direct or indirect interaction of PrPC with extracellular matrix proteins, soluble factors, transmembrane proteins, G-protein coupled receptors and ions channels. The ability of PrPC to drive the assembly of multi-component complexes at the cell surface is likely the basis for its neurotrophic functions. These properties indicate that PrPC may be relevant for not only the spongiform encephalophaties, but also as an ancillary component of the pathogenesis of other neurodegenerative diseases, and therefore amenable to therapeutic targeting.
Keywords: PrPC; neurotrophic; spongiform encephalophaties; neurodegenerative diseases; therapeutic targeting PrPC; neurotrophic; spongiform encephalophaties; neurodegenerative diseases; therapeutic targeting

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MDPI and ACS Style

Martins, V.R.; Beraldo, F.H.; Hajj, G.N.; Lopes, M.H.; Lee, K.S.; Prado, M.A.; Linden, R. Prion Protein: Orchestrating Neurotrophic Activities. Curr. Issues Mol. Biol. 2010, 12, 63-86. https://doi.org/10.21775/cimb.012.063

AMA Style

Martins VR, Beraldo FH, Hajj GN, Lopes MH, Lee KS, Prado MA, Linden R. Prion Protein: Orchestrating Neurotrophic Activities. Current Issues in Molecular Biology. 2010; 12(2):63-86. https://doi.org/10.21775/cimb.012.063

Chicago/Turabian Style

Martins, Vilma R., Flavio H. Beraldo, Glaucia N. Hajj, Marilene H. Lopes, Kil Sun Lee, Marco A. Prado, and Rafael Linden. 2010. "Prion Protein: Orchestrating Neurotrophic Activities" Current Issues in Molecular Biology 12, no. 2: 63-86. https://doi.org/10.21775/cimb.012.063

APA Style

Martins, V. R., Beraldo, F. H., Hajj, G. N., Lopes, M. H., Lee, K. S., Prado, M. A., & Linden, R. (2010). Prion Protein: Orchestrating Neurotrophic Activities. Current Issues in Molecular Biology, 12(2), 63-86. https://doi.org/10.21775/cimb.012.063

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