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The prion protein family
Authors:Bruno Passet  Sophie Halliez  Vincent Béringue  Hubert Laude  Jean-Luc Vilotte
Affiliation:1.UMR1313 Génétique Animale et Biologie Intégrative; Institut Nationale de la Recherche Agronomique; Jouy-en-Josas, France;2.UR892 Virologie et Immunologie Moléculaires; Institut Nationale de la Recherche Agronomique; Jouy-en-Josas, France
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.
Keywords:Shadoo  doppel  prion  PrP  embryo  placenta  trophectoderm  stem cell
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