AUTHOR=Sakudo Akikazu , Onodera Takashi 

TITLE=Prion protein (PrP) gene-knockout cell lines: insight into functions of the PrP

JOURNAL=Frontiers in Cell and Developmental Biology

VOLUME=2

YEAR=2015

URL=https://www.frontiersin.org/journals/cell-and-developmental-biology/articles/10.3389/fcell.2014.00075

DOI=10.3389/fcell.2014.00075

ISSN=2296-634X

ABSTRACT=<p>Elucidation of prion protein (PrP) functions is crucial to fully understand prion diseases. A major approach to studying PrP functions is the use of PrP gene-knockout (<italic>Prnp</italic><sup>−/−</sup>) mice. So far, six types of <italic>Prnp</italic><sup>−/−</sup> mice have been generated, demonstrating the promiscuous functions of PrP. Recently, other PrP family members, such as Doppel and Shadoo, have been found. However, information obtained from comparative studies of structural and functional analyses of these PrP family proteins do not fully reveal PrP functions. Recently, varieties of <italic>Prnp</italic><sup>−/−</sup> cell lines established from <italic>Prnp</italic><sup>−/−</sup> mice have contributed to the analysis of PrP functions. In this mini-review, we focus on <italic>Prnp</italic><sup>−/−</sup> cell lines and summarize currently available <italic>Prnp</italic><sup>−/−</sup> cell lines and their characterizations. In addition, we introduce the recent advances in the methodology of cell line generation with knockout or knockdown of the PrP gene. We also discuss how these cell lines have provided valuable insights into PrP functions and show future perspectives.</p>