Frontiers in Cell and Developmental Biology (Oct 2014)
Role of the prion protein family in the gonads
Abstract
The prion-gene family comprises 4 members named PRNP (PRPc), PRND (Doppel), PRNT(PRT) and SPRN (Shadoo). According to species, PRND is located 16-52 kb downstreamfrom the PRNP locus, whereas SPRN is located on another chromosome. The fourth prionfamilygene, PRNT, belongs to the same genomic cluster as PRNP and PRND in humans andbovidae. PRNT and PRND possibly resulted from a duplication event of PRND and PRNP,respectively, that occurred early during eutherian species divergence. Although most of thestudies concerning the prion-family has been done on PRPc and its involvement intransmissible neurodegenerative disorders, different works report some potential roles ofthese proteins in the reproductive function of both sexes. Among them, a clear role of PRND,that encodes for the Doppel protein, in male fertility has been demonstrated through genetargeting studies in mice. In other species, Doppel seems to play a role in testis and ovarydevelopment but its cellular localization is variable according to the gonadal developmentalstage and to the mammalian species considered. For the other three genes, their roles inreproductive function appear ill-defined and/or controversial. The present review aimed tosynthesize all the available data on these prion-family members and their relations withreproductive processes, mainly in the gonad of both sexes.
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