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Molecular Neurobiology

, Volume 49, Issue 1, pp 413–423 | Cite as

Gene Expression Resulting from PrPC Ablation and PrPC Overexpression in Murine and Cellular Models

  • Franc Llorens
  • Isidre Ferrer
  • José Antonio del Río
Article

Abstract

The cellular prion protein (PrPC) plays a key role in prion diseases when it converts to the pathogenic form scrapie prion protein. Increasing knowledge of its participation in prion infection contrasts with the elusive and controversial data regarding its physiological role probably related to its pleiotropy, cell-specific functions, and cellular-specific milieu. Multiple approaches have been made to the increasing understanding of the molecular mechanisms and cellular functions modulated by PrPC at the transcriptomic and proteomic levels. Gene expression analyses have been made in several mouse and cellular models with regulated expression of PrPC resulting in PrPC ablation or PrPC overexpression. These analyses support previous functional data and have yielded clues about new potential functions. However, experiments on animal models have shown moderate and varied results which are difficult to interpret. Moreover, studies in cell cultures correlate little with in vivo counterparts. Yet, both animal and cell models have provided some insights on how to proceed in the future by using more refined methods and selected functional experiments.

Keywords

Gene expression Prion protein Microarrays Deep sequencing RNA-seq Gene knockout Neurodegeneration 

Abbreviations

PrPC

Cellular prion protein

PrPSc

Scrapie prion protein

CNS

Central nervous system

Notes

Acknowledgments

The authors are deeply grateful to present and past members of our laboratory who contributed to gene expression experiments and to Dr. Lauro Sumoy for manuscript supervision. This study was funded by the Seventh Framework Programme of the European Commission, grant agreement 222887, FP7 PRIORITY, DEMTEST, and grant agreement 278486, DEVELAGE (Joint Programme on Neurodegenerative Diseases, PI11/03028); the Spanish Ministry of Science and Innovation (MINECO, BFU2012-32617); the Generalitat de Catalunya (SGR2009-366); La Caixa Obra Social Foundation; and the Carlos III Health Institute.

Conflict of Interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Franc Llorens
    • 1
    • 2
  • Isidre Ferrer
    • 1
    • 2
    • 5
  • José Antonio del Río
    • 2
    • 3
    • 4
  1. 1.Institute of NeuropathologyUniversity Hospital Bellvitge—Bellvitge Biomedical Research Institute (IDIBELL)L’Hospitalet de LlobregatSpain
  2. 2.Network Biomedical Research Center for Neurodegenerative Diseases (CIBERNED)MadridSpain
  3. 3.Institute for Bioengineering of Catalonia (IBEC)BarcelonaSpain
  4. 4.Department of Cell BiologyUniversity of Barcelona (UB)BarcelonaSpain
  5. 5.Department of Pathology and Experimental TherapeuticsUniversity of Barcelona (UB)L’Hospitalet de LlobregatSpain

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