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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Journal ArticleDOI
Cell mediated immune responses against human prion protein.
J. Bainbridge,B. Walker +1 more
TL;DR: This study examines active PrP specific T cell responses that can be produced in PrP null (PrP 0/0) mice using simple peptide vaccination procedures and indicates the stimulation of a heterogenous population of T cells with an increase in cytokines and cytotoxicity associated mRNA.
Journal ArticleDOI
Human prion protein-induced autophagy flux governs neuron cell damage in primary neuron cells
Ji-Hong Moon,Ju-Hee Lee,Uddin Md Nazim,You-Jin Lee,Jae-Won Seol,Seong-Kug Eo,John-Hwa Lee,Sang-Youel Park +7 more
TL;DR: It is demonstrated that prion protein-induced autophagy flux is involved in neuron cell death in prion disease and suggest that autophagic flux might play a critical role in neurodegenerative diseases including prions disease.
Journal ArticleDOI
Identification of adjacent binding sites for the YY1 and E4BP4 transcription factors in the ovine PrP (Prion) gene promoter.
Stewart T. G. Burgess,Cuicui Shen,Cuicui Shen,Laura A. Ferguson,Gerard T. O’Neill,Kevin Docherty,Nora Hunter,Wilfred Goldmann +7 more
TL;DR: The PrP gene encodes the cellular isoform of the prion protein (PrPc) which has been shown to be crucial to the development of transmissible spongiform encephalopathies (TSEs) and the distribution of TSE infectivity may be associated with PrP promoter genotype.
This Provisional PDF corresponds to the article as it appeared upon acceptance. Fully formatted PDF and full text (HTML) versions will be made available soon. Brain transcriptional stability upon prion protein-encoding gene invalidation in zygotic or adult mouse.
Rachel Young,Sandrine Le Guillou,Frédérique Bitton,Marie-Laure Martin-Magniette,Vincent Beringue,Fabienne Le Provost,Jean-Luc Vilotte +6 more
TL;DR: The results suggest that the physiological function of PrP is redundant at the adult stage or important for only a small subset of the brain cell population under classical breeding conditions.
Journal ArticleDOI
Molecular Biology of Prion Protein and Its First Homologous Protein
TL;DR: No neuronal cell death could be detected in PrP-/- mice, indicating that the functional loss of PrP(C) alone might not be enough to induce neuronal cellDeath, one of major pathological hallmarks of prion diseases.
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.