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
Bifunctional roles for two topological isoforms of the cellular prion protein
TL;DR: Evidence is provided that wild type PrPC has the intrinsic capacity to be expressed as either SecPrP or CtmPrP and that each isoform can partake in independent pathways.
DissertationDOI
Development and Characterisation of PC12 Cell Lines Allowing Inducible Expression of Prion Proteins Carrying Pathogenic Mutations
TL;DR: A tetracycline-inducible (Tet-on) model was developed, in which PrP expression is switched on after clonal selection, and it is demonstrated that expression of PrP does not protect PC 12 Tet-on cells from such stresses.
Journal ArticleDOI
Rôle du système immunitaire dans les encéphalopathies spongiformes subaiguës transmissibles ou maladies à prions
TL;DR: Les encephalopathies spongiformes subaigues transmissibles se presentent comme des maladies neurodegeneratives du systeme nerveux central, sans syndrome inflammatoire associe, avec un double determinisme genetique et infectieux dans la physiopathologie des ESSTs.
Journal ArticleDOI
Bona fide atypical scrapie faithfully reproduced for the first time in a rodent model
Enric Vidal,Manuel Sánchez-Martín,Hasier Eraña,Sonia Pérez Lázaro,Miguel A. Pérez-Castro,Alicia Otero,Jorge M. Charco,Belén Marín,Rafael Lopez-Moreno,Carlos M. Díaz-Domínguez,Mariví Geijo,Montserrat Ordóñez,Guillermo Cantero,Michele Angelo Di Bari,Nuria L. Lorenzo,Laura Pirisinu,Claudia D'Agostino,Juan María Torres,Vincent Béringue,Glenn C. Telling,Juan José Badiola,Martí Pumarola,Rosa Bolea,Romolo Nonno,Jesús R. Requena,Joaquín Castilla +25 more
TL;DR: In this article , the authors presented the first faithful model for a bona fide, transmissible, ovine, atypical scrapie prion disease in a transgenic mouse model.
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.