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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Gene expression profile following stable expression of the cellular prion protein.
Jun-ichi Satoh,Takashi Yamamura +1 more
TL;DR: Results indicate that accumulation of PrPC in the cell caused aberrant regulation of a battery of the genes important for specific neuronal function, which represents a possible mechanism underlying PrPC-mediated selective neurodegeneration.
Journal ArticleDOI
Molecular modulation of expression of prion protein by heat shock.
Woei-Cherng Shyu,Woei-Cherng Shyu,Horng-Jyh Harn,Horng-Jyh Harn,Keiichi Saeki,Astsutaka Kubosaki,Yoshitsugu Matsumoto,Takashi Onodera,Cheng-Jueng Chen,Cheng-Jueng Chen,Yaw-Don Hsu,Yung-Hsiao Chiang +11 more
TL;DR: Results suggest that cellular stress up-regulates both the transcription and translation of PrP through interaction with the HSEs on the PrP gene promoter, resulting in an increase in protein synthesis.
Journal ArticleDOI
The Glycosylation Status of PrPC Is a Key Factor in Determining Transmissible Spongiform Encephalopathy Transmission between Species
Frances K. Wiseman,Enrico Cancellotti,Pedro Piccardo,Kayleigh Iremonger,Aileen Boyle,Deborah Brown,James W. Ironside,Jean Manson,Abigail B. Diack +8 more
TL;DR: It is shown in vivo that glycosylation of the host PrPC has a significant impact on the transmission of TSE between different host species, highlighting glycosolation of PrPC as a key factor in determining the transmission efficiency of T SEs between different species.
Journal ArticleDOI
Different Behavior toward Bovine Spongiform Encephalopathy Infection of Bovine Prion Protein Transgenic Mice with One Extra Repeat Octapeptide Insert Mutation
Joaquín Castilla,Alfonso Gutiérrez-Adán,Alejandro Brun,Belén Pintado,B. Parra,Miguel Ángel Ramírez,Francisco J. Salguero,F. Díaz San Segundo,A. Rábano,María J. Cano,Juan María Torres +10 more
TL;DR: Transgenic mice expressing bovine PrP bearing an additional octapeptide insertion to the wild type showed an altered course of bovines spongiform encephalopathy (BSE) infection, reflected as reduced incubation times when compared with boTg mice expressing similar levels of the wild-type six-octapeptides.
Journal ArticleDOI
Application of a novel in vitro selection technique to isolate and characterise high affinity DNA aptamers binding mammalian prion proteins.
David F. Bibby,Andrew C. Gill,Louise Kirby,Christine Farquhar,Moira E. Bruce,Jeremy A. Garson +5 more
TL;DR: Preliminary studies indicate that a trivalent aptamer pool is capable of binding the pathological isoform PrP(Sc) following guanidinium denaturation, and this work applies SELEX against a recombinant murine prion protein to select single-stranded DNA ligands (aptamers) of high affinity.
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.