title: The prion or the related Shadoo protein is required for early mouse embryogenesis.
abstract: The prion protein PrP has a key role in transmissible spongiform encephalopathies but its biological function remains largely unknown. Recently, a related protein, Shadoo, was discovered. Its biological properties and brain distribution partially overlap that of PrP. We report that the Shadoo-encoding gene knockdown in PrP-knockout mouse embryos results in a lethal phenotype, occurring between E8 and E11, not observed on the wild-type genetic background. It reveals that these two proteins play a shared, crucial role in mammalian embryogenesis, explaining the lack of severe phenotype in PrP-knockout mammals, an appreciable step towards deciphering the biological role of this protein family.
authors.affiliation: INRA-UMR1313, Génétique Animale et Biologie Intégrative, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France.
authors.forename: Rachel
authors.initials: R
authors.lastname: Young
authors.forename: Bruno
authors.initials: B
authors.lastname: Passet
authors.forename: Marthe
authors.initials: M
authors.lastname: Vilotte
authors.forename: Edmond P
authors.initials: EP
authors.lastname: Cribiu
authors.forename: Vincent
authors.initials: V
authors.lastname: Béringue
authors.forename: Fabienne
authors.initials: F
authors.lastname: Le Provost
authors.forename: Hubert
authors.initials: H
authors.lastname: Laude
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Vilotte
mesh_terms: D000818:Animals; D015536:Down-Regulation; D047108:Embryonic Development / Q000235:genetics*; D058851:GPI-Linked Proteins; D055785:Gene Knockdown Techniques; D005804:Genes, Lethal; D016086:Lentivirus; D051379:Mice; D018345:Mice, Knockout; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism*; D011328:Prions / Q000235:genetics / Q000378:metabolism*
chemical_list: D058851:GPI-Linked Proteins; D009419:Nerve Tissue Proteins; D011328:Prions; C523856:Sprn protein, mouse