AUTHOR=Allais-Bonnet Aurélie , Pailhoux Eric 

TITLE=Role of the prion protein family in the gonads

JOURNAL=Frontiers in Cell and Developmental Biology

VOLUME=2

YEAR=2014

URL=https://www.frontiersin.org/journals/cell-and-developmental-biology/articles/10.3389/fcell.2014.00056

DOI=10.3389/fcell.2014.00056

ISSN=2296-634X

ABSTRACT=<p>The prion-gene family comprises four members named <italic>PRNP</italic> (PRP<sup>c</sup>), <italic>PRND</italic> (Doppel), <italic>PRNT</italic> (PRT), and <italic>SPRN</italic> (Shadoo). According to species, <italic>PRND</italic> is located 16–52 kb downstream from the <italic>PRNP</italic> locus, whereas <italic>SPRN</italic> is located on another chromosome. The fourth prion-family gene, <italic>PRNT</italic>, belongs to the same genomic cluster as <italic>PRNP</italic> and <italic>PRND</italic> in humans and bovidae. <italic>PRNT</italic> and <italic>PRND</italic> possibly resulted from a duplication event of <italic>PRND</italic> and <italic>PRNP</italic>, respectively, that occurred early during eutherian species divergence. Although most of the studies concerning the prion-family has been done on PRP<sup>c</sup> and its involvement in transmissible neurodegenerative disorders, different works report some potential roles of these proteins in the reproductive function of both sexes. Among them, a clear role of <italic>PRND</italic>, that encodes for the Doppel protein, in male fertility has been demonstrated through gene targeting studies in mice. In other species, Doppel seems to play a role in testis and ovary development but its cellular localization is variable according to the gonadal developmental stage and to the mammalian species considered. For the other three genes, their roles in reproductive function appear ill-defined and/or controversial. The present review aimed to synthesize all the available data on these prion-family members and their relations with reproductive processes, mainly in the gonad of both sexes.</p>