title: To develop with or without the prion protein.
abstract: The deletion of the cellular form of the prion protein (PrP(C)) in mouse, goat, and cattle has no drastic phenotypic consequence. This stands in apparent contradiction with PrP(C) quasi-ubiquitous expression and conserved primary and tertiary structures in mammals, and its pivotal role in neurodegenerative diseases such as prion and Alzheimer's diseases. In zebrafish embryos, depletion of PrP ortholog leads to a severe loss-of-function phenotype. This raises the question of a potential role of PrP(C) in the development of all vertebrates. This view is further supported by the early expression of the PrP(C) encoding gene (Prnp) in many tissues of the mouse embryo, the transient disruption of a broad number of cellular pathways in early Prnp(-/-) mouse embryos, and a growing body of evidence for PrP(C) involvement in the regulation of cell proliferation and differentiation in various types of mammalian stem cells and progenitors. Finally, several studies in both zebrafish embryos and in mammalian cells and tissues in formation support a role for PrP(C) in cell adhesion, extra-cellular matrix interactions and cytoskeleton. In this review, we summarize and compare the different models used to decipher PrP(C) functions at early developmental stages during embryo- and organo-genesis and discuss their relevance.
authors.affiliation: Institut National de la Recherche Agronomique, U892 Virologie et Immunologie Moléculaires Jouy-en-Josas, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Halliez
authors.affiliation: Institut National de la Recherche Agronomique, UMR1313 Génétique Animale et Biologie Intégrative Jouy-en-Josas, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Passet
authors.affiliation: Institut National de la Santé et de la Recherche Médicale, UMR-S1124 Paris, France ; Université Paris Descartes, Sorbonne Paris Cité, UMR-S1124 Paris, France.
authors.forename: Séverine
authors.initials: S
authors.lastname: Martin-Lannerée
authors.affiliation: Institut National de la Santé et de la Recherche Médicale, UMR-S1124 Paris, France ; Université Paris Descartes, Sorbonne Paris Cité, UMR-S1124 Paris, France.
authors.forename: Julia
authors.initials: J
authors.lastname: Hernandez-Rapp
authors.affiliation: Institut National de la Recherche Agronomique, U892 Virologie et Immunologie Moléculaires Jouy-en-Josas, France.
authors.forename: Hubert
authors.initials: H
authors.lastname: Laude
authors.affiliation: Institut National de la Santé et de la Recherche Médicale, UMR-S1124 Paris, France ; Université Paris Descartes, Sorbonne Paris Cité, UMR-S1124 Paris, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Mouillet-Richard
authors.affiliation: Institut National de la Recherche Agronomique, UMR1313 Génétique Animale et Biologie Intégrative Jouy-en-Josas, France.
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Vilotte
authors.affiliation: Institut National de la Recherche Agronomique, U892 Virologie et Immunologie Moléculaires Jouy-en-Josas, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Béringue
keywords: cell adhesion; cytoskeleton; development; extra-cellular matrix; neural development; prion protein; stem cells