Document Detail


The prion protein family: Looking outside the central nervous system.
MedLine Citation:
PMID:  23154632     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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:
Bruno Passet; Sophie Halliez; Vincent Béringue; Hubert Laude; Jean-Luc Vilotte
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-15
Journal Detail:
Title:  Prion     Volume:  7     ISSN:  1933-690X     ISO Abbreviation:  Prion     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101472305     Medline TA:  Prion     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
UMR1313 Génétique Animale et Biologie Intégrative; Institut Nationale de la Recherche Agronomique; Jouy-en-Josas, France.
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