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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Prion protein as a receptor for amyloid-beta oligomers
TL;DR: In this article, the authors proposed methods of inhbiting suppression of long term potentiation, improving acute memory retention and spatial memory performance and treating Alzheimer's disease by administration of a PrPc antagonist or combinations thereof.
Journal ArticleDOI
In Vitro Approach To Identify Key Amino Acids in Low Susceptibility of Rabbit Prion Protein to Misfolding.
Hasier Eraña,Natalia Fernández-Borges,Saioa R. Elezgarai,Chafik Harrathi,Jorge M. Charco,Francesca Chianini,Mark P. Dagleish,Gabriel Ortega,Oscar Millet,Joaquín Castilla +9 more
TL;DR: Using recombinant rabbit PrP as a model, critical molecular determinants that confer this high resistance to transmissible spongiform encephalopathies are described.
Journal ArticleDOI
Prions: pathogenesis and reverse genetics.
Adriano Aguzzi,Michael A. Klein,Fabio Montrasio,Vladimir Pekarik,Sebastian Brandner,Hisako Furukawa,Pascal Käser,Christiane Röckl,Markus Glatzel +8 more
TL;DR: Ectopic expression of PrPc inPrPc knockout mice proved a useful tool for the identification of host cells competent for prion replication and selective reconstitution experiments aimed at expressing PrPC in neurografts or in specific populations of hemato‐ and lymphopoietic cells helped in elucidating some of the mechanisms of prion spread and disease pathogenesis.
Journal ArticleDOI
Prion protein (PrP) gene-knockout cell lines: insight into functions of the PrP.
Akikazu Sakudo,Takashi Onodera +1 more
TL;DR: This mini-review focuses on Prnp−/− cell lines and summarizes currently available Prnp+/−cell lines and their characterizations, and introduces the recent advances in the methodology of cell line generation with knockout or knockdown of the PrP gene.
Journal ArticleDOI
Dysfunction and recovery of synapses in prion disease: implications for neurodegeneration
TL;DR: Mice with prion disease can be cured at the stage of early synaptic dysfunction, when they have reversible impairments at neurophysiological, behavioural and morphological levels, and reversing synaptic dysfunction at this stage of disease rescues neurons, preventing its otherwise inevitable progression to synapse loss and cell death.
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.