title: The prion protein family: looking outside the central nervous system.
abstract: Although the pivotal implication of the host-encoded Prion protein, PrP, in the neuropathology of transmissible spongiform encephalopathy is known for decades, its biological role remains mostly elusive. Genetic inactivation is one way to assess such issue but, so far, PrP-knockout mice did not help much. However, recent reports involving (1) further studies of these mice during embryogenesis, (2) knockdown experiments in Zebrafish and (3) knockdown of Shadoo, a protein with PrP-like functional domains, in PrP-knockout mice, all suggested a role of the Prion protein family in early embryogenesis. This view is challenged by the recent report that PrP/Shadoo knockout mice are healthy and fertile. Although puzzling, these apparently contradictory data may on the contrary help at deciphering the Prion protein family role through focusing scientific attention outside the central nervous system and by helping the identification of other loci involved in the genetic robustness associated with PrP.
authors.affiliation: UMR1313 Génétique Animale et Biologie Intégrative, Institut Nationale de la Recherche Agronomique, Jouy-en-Josas, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Passet
authors.forename: Sophie
authors.initials: S
authors.lastname: Halliez
authors.forename: Vincent
authors.initials: V
authors.lastname: Béringue
authors.forename: Hubert
authors.initials: H
authors.lastname: Laude
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Vilotte
mesh_terms: D000818:Animals; D047108:Embryonic Development; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D009419:Nerve Tissue Proteins / Q000378:metabolism; D011328:Prions / Q000378:metabolism*
chemical_list: D009419:Nerve Tissue Proteins; D011328:Prions