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
Role of polymorphic variants of the prion protein on the resistance-susceptibility to prion infection
TL;DR: In this paper, a panel of lineas of raton transgenico (Tg) that expresan the PrPC de cabra de tipo salvaje (wt) or variantes of the prionica patogena debidas a cambios puntuales de aa (I/M142, R/H154), R/Q211, Q/K222, Q /R171 and N/K176).
Dissertation
Characterization of the 37-kDa/67-kDa laminin receptor as the cell surface receptor for the cellular prion protein
TL;DR: The possible role of LRP/LR as a cell surface receptor for PrPc, which represents the precursor of the human 67 kDa high-affinity laminin receptor precursor (LR), was identified as a binding partner for the cellular prion protein in a yeast two-hybrid screen.
Single chain antibodies against the 37 kDa/67 kDa laminin receptor as tools for prion diseases therapy
TL;DR: Results indicate that LRP is not only required for PrPc metabolism under non-pathological conditions but also has a pivotal role in prion propagation in a cell culture model.
Journal ArticleDOI
Spatial and temporal down-regulation of transgene expression using the TRSID-silencer in mice: application to Prnp.
Micaela Gallozzi,Vincent Béringue,Pauline Decaunes,Annick Le Dur,Karine Le Roux,Gaëlle Tilly,Sandrine Guillou,Laetitia Herzog,Coralie Peyre,Aline Ladroue,Jérôme Chapuis,Marthe Vilotte,Bruno Passet,José Costa,Nathalie Chenais,Fabienne Le Provost,Hubert Laude,Jean-Luc Vilotte +17 more
TL;DR: In bi‐transgenic mice, ovine PrPC expression could be reversibly controlled in neuronal cells by doxycycline treatment whereas it remains constant in other cell types while the involvement of cell types in prion diseases and PrP physiological function is assessed.
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.