Journal ArticleDOI
129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal.
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TLDR
The use of a different targeting strategy is reported, to produce inbred mice with a complete absence of both PrP protein and mRNA sequences, which are being used in experiments designed to address the role of PrP in the pathogenesis of scrapie and the replication of infectivity.Abstract:
The neural membrane glycoprotein PrP is implicated in the pathogenesis of the transmissible spongiform encephalopathies; however, the normal function of PrP and its precise role in disease are not understood. Recently, gene targeting has been used to produce mice withneo/PrP fusion transcripts, but no detectable PrP protein in the brain (1). Here we report the use of a different targeting strategy, to produce inbred mice with a complete absence of both PrP protein and mRNA sequences. At 7 mo of age, these mice show no overt phenotypic abnormalities despite the normal high levels of expression of PrP during mouse development. The mice are being used in experiments designed to address the role of PrP in the pathogenesis of scrapie and the replication of infectivity.read more
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Dissertation
Rôle des cellules immunitaires et effet des cannabinoïdes dans la physiopathologie des maladies à prions
TL;DR: The role physiologique de ces interactions and the nature moleculaire des recepteurs reste a etre determines as discussed by the authors, en particulier dans le systeme immunitaire.
Pharmacological and genetic manipulation of autophagy and its impact on diverse prion infection scenarios
TL;DR: This study provides the direct experimental evidence for the impact of autophagy in prion disease and identifies the drug tamoxifen as well as the gene beclin 1 as starting points for the development of novel therapies.
Journal ArticleDOI
Epigenetic Control of the Notch and Eph Signaling Pathways by the Prion Protein: Implications for Prion Diseases
Théo Z. Hirsch,Théo Z. Hirsch,Séverine Martin-Lannerée,Séverine Martin-Lannerée,Fabienne Reine,Julia Hernandez-Rapp,Julia Hernandez-Rapp,Julia Hernandez-Rapp,Laetitia Herzog,Michel Dron,Nicolas Privat,Nicolas Privat,Bruno Passet,Sophie Halliez,Sophie Halliez,Ana Villa-Diaz,Caroline Lacroux,Victor Klein,Victor Klein,Stéphane Haïk,Stéphane Haïk,Olivier Andreoletti,Juan María Torres,Jean-Luc Vilotte,Vincent Beringue,Sophie Mouillet-Richard,Sophie Mouillet-Richard +26 more
TL;DR: It is concluded that infectious prions cause a loss-of-function phenotype with respect to Notch and Eph signaling and that these alterations are sustained by epigenetic mechanisms.
Journal ArticleDOI
Expression of the prion-like protein Shadoo in the developing mouse embryo.
Rachel Young,Stephan Bouet,Jacqueline Polyte,Sandrine Guillou,Bruno Passet,Marthe Vilotte,Johan Castille,Vincent Béringue,Fabienne Le Provost,Hubert Laude,Jean Luc Vilotte +10 more
TL;DR: The observed pattern in specific embryonic cell lineages and in extra-embryonic tissues is consistent with the previously reported phenotype resulting from its knockdown.
Journal ArticleDOI
Transgenic mouse models expressing human and macaque prion protein exhibit similar prion susceptibility on a strain-dependent manner
Juan Carlos Espinosa,Emmanuel Comoy,Alba Marín-Moreno,Patricia Aguilar-Calvo,Marie-Christine Birling,José Luis Pitarch,Jean-Philippe Deslys,Juan María Torres +7 more
TL;DR: Interestingly, TgMac were more susceptible to classical-BSE strain infection than Hu-Tg340, suggesting that macaque is an adequate model for the evaluation of human susceptibility to most of the prion strains tested.
References
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Journal ArticleDOI
Single-step method of RNA isolation by acid guanidinium thiocyanate-phenol-chloroform extraction
TL;DR: A new method of total RNA isolation by a single extraction with an acid guanidinium thiocyanate-phenol-chloroform mixture is described, providing a pure preparation of undegraded RNA in high yield and can be completed within 4 h.
Journal ArticleDOI
Mice deficient for p53 are developmentally normal but susceptible to spontaneous tumours
Lawrence A. Donehower,Michele Harvey,Betty L. Slagle,Mark J. McArthur,Charles A. Montgomery,Janet S. Butel,Allan Bradley +6 more
TL;DR: Observations indicate that a normal p53 gene is dispensable for embryonic development, that its absence predisposes the animal to neoplastic disease, and that an oncogenic mutant form of p53 is not obligatory for the genesis of many types of tumours.
Journal ArticleDOI
Site-directed mutagenesis by gene targeting in mouse embryo-derived stem cells.
Kirk R. Thomas,Mario R. Capecchi +1 more
TL;DR: This work mutated, by gene targeting, the endogenous hypoxanthine phosphoribosyl transferase (HPRT) gene in mouse embryo-derived stem (ES) cells and compared the gene-targeting efficiencies of two classes of neor-Hprt recombinant vectors.
Journal ArticleDOI
Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein
Hansruedi Büeler,Marek Fischer,Yolande Lang,Yolande Lang,Horst Bluethmann,Horst Bluethmann,Hans-Peter Lipp,Stephen J. DeArmond,Stephen J. DeArmond,Stanley B. Prusiner,Stanley B. Prusiner,Michel Aguet,Charles Weissmann +12 more
TL;DR: It is now feasible to determine whether mice devoid of PrPc can propagate prions and are susceptible to scrapie pathogenesis.
Journal ArticleDOI
Multiple intestinal neoplasia caused by a mutation in the murine homolog of the APC gene.
Li Kuo Su,Kenneth W. Kinzler,Bert Vogelstein,Antoinette C. Preisinger,Amy R. Moser,Cindy Luongo,Karen A. Gould,William F. Dove +7 more
TL;DR: In this paper, a mouse lineage that exhibits an autosomal dominantly inherited predisposition to multiple intestinal neoplasia (Min) was described and linkage analysis showed that the murine homolog of the APC gene (mApc) was tightly linked to the Min locus.