To Develop With Or Without The Prion Protein

The deletion of the cellular form of the prion protein (PrPC) in mouse, goat and cattle has no drastic phenotypic consequence. This stands in apparent contradiction with PrPC quasi- ubiquitous expression and conserved primary and tertiary structures in mammals, and its pivotal role in neurodegenerat...

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Main Authors: Sophie eHalliez, Bruno ePasset, Séverine eMartin-Lannerée, Julia eHernandez-Rapp, Hubert eLaude, Sophie eMouillet-Richard, Jean-Luc eVilotte, Vincent eBéringue
Format: Article
Language:English
Published: Frontiers Media S.A. 2014-10-01
Series:Frontiers in Cell and Developmental Biology
Subjects:
Online Access:http://journal.frontiersin.org/Journal/10.3389/fcell.2014.00058/full
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author Sophie eHalliez
Bruno ePasset
Séverine eMartin-Lannerée
Séverine eMartin-Lannerée
Julia eHernandez-Rapp
Julia eHernandez-Rapp
Hubert eLaude
Sophie eMouillet-Richard
Sophie eMouillet-Richard
Jean-Luc eVilotte
Vincent eBéringue
author_facet Sophie eHalliez
Bruno ePasset
Séverine eMartin-Lannerée
Séverine eMartin-Lannerée
Julia eHernandez-Rapp
Julia eHernandez-Rapp
Hubert eLaude
Sophie eMouillet-Richard
Sophie eMouillet-Richard
Jean-Luc eVilotte
Vincent eBéringue
author_sort Sophie eHalliez
collection DOAJ
description The deletion of the cellular form of the prion protein (PrPC) in mouse, goat and cattle has no drastic phenotypic consequence. This stands in apparent contradiction with PrPC 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 PrPC in the development of all vertebrates. This view is further supported by the early expression of the PrPC 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 PrPC 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 PrPC in cell adhesion, extra-cellular matrix interactions and cytoskeleton. In this review, we summarize and compare the different models used to decipher PrPC functions at early developmental stages during embryo- and organo- genesis and discuss their relevance.
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spelling doaj.art-9c7c726243d14f8f93c65d97106d4efe2022-12-22T03:30:03ZengFrontiers Media S.A.Frontiers in Cell and Developmental Biology2296-634X2014-10-01210.3389/fcell.2014.00058111417To Develop With Or Without The Prion ProteinSophie eHalliez0Bruno ePasset1Séverine eMartin-Lannerée2Séverine eMartin-Lannerée3Julia eHernandez-Rapp4Julia eHernandez-Rapp5Hubert eLaude6Sophie eMouillet-Richard7Sophie eMouillet-Richard8Jean-Luc eVilotte9Vincent eBéringue10INRAINRAINSERMUniversité Paris Descartes, Sorbonne Paris CitéINSERMUniversité Paris Descartes, Sorbonne Paris CitéINRAINSERMUniversité Paris Descartes, Sorbonne Paris CitéINRAINRAThe deletion of the cellular form of the prion protein (PrPC) in mouse, goat and cattle has no drastic phenotypic consequence. This stands in apparent contradiction with PrPC 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 PrPC in the development of all vertebrates. This view is further supported by the early expression of the PrPC 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 PrPC 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 PrPC in cell adhesion, extra-cellular matrix interactions and cytoskeleton. In this review, we summarize and compare the different models used to decipher PrPC functions at early developmental stages during embryo- and organo- genesis and discuss their relevance.http://journal.frontiersin.org/Journal/10.3389/fcell.2014.00058/fullCell AdhesionCytoskeletonStem Cellsdevelopmentprion proteinneural development
spellingShingle Sophie eHalliez
Bruno ePasset
Séverine eMartin-Lannerée
Séverine eMartin-Lannerée
Julia eHernandez-Rapp
Julia eHernandez-Rapp
Hubert eLaude
Sophie eMouillet-Richard
Sophie eMouillet-Richard
Jean-Luc eVilotte
Vincent eBéringue
To Develop With Or Without The Prion Protein
Frontiers in Cell and Developmental Biology
Cell Adhesion
Cytoskeleton
Stem Cells
development
prion protein
neural development
title To Develop With Or Without The Prion Protein
title_full To Develop With Or Without The Prion Protein
title_fullStr To Develop With Or Without The Prion Protein
title_full_unstemmed To Develop With Or Without The Prion Protein
title_short To Develop With Or Without The Prion Protein
title_sort to develop with or without the prion protein
topic Cell Adhesion
Cytoskeleton
Stem Cells
development
prion protein
neural development
url http://journal.frontiersin.org/Journal/10.3389/fcell.2014.00058/full
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