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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The Cellular Prion Protein (PrPC): Its Physiological Function and Role in Disease
TL;DR: Some of the major proposed functions for PrP(C), including protection against apoptotic and oxidative stress, cellular uptake or binding of copper ions, transmembrane signaling, formation and maintenance of synapses, and adhesion to the extracellular matrix are summarized.
Journal ArticleDOI
Axonal prion protein is required for peripheral myelin maintenance
Juliane Bremer,Frank Baumann,Cinzia Tiberi,Carsten Wessig,Heike Fischer,Petra Schwarz,Andrew D. Steele,Klaus V. Toyka,Klaus-Armin Nave,Joachim Weis,Adriano Aguzzi +10 more
TL;DR: It is shown that ablation of the prion protein PrPC triggers a chronic demyelinating polyneuropathy in four independently targeted mouse strains, indicating that neuronal expression and regulated proteolysis of PrPC are essential for myelin maintenance.
Journal ArticleDOI
Porphyrin and phthalocyanine antiscrapie compounds.
TL;DR: Treatment of TSE-infected animals with three structurally dissimilar tetrapyrroles identifies these compounds as anti-TSE drugs and increases survival time from 50 to 300%.
Journal ArticleDOI
Production of cattle lacking prion protein.
Jiirgen A. Richt,Poothappillai Kasinathan,Amir N. Hamir,Joaquín Castilla,Thillai Sathiyaseelan,Francisco Vargas,Janaki Sathiyaseelan,Hua Wu,Hiroaki Matsushita,Julie Koster,Shinichiro Kato,Isao Ishida,Claudio Soto,James M. Robl,Yoshimi Kuroiwa +14 more
TL;DR: The generation and characterization of PrPC-deficient cattle produced by a sequential gene-targeting system are reported and at over 20 months of age the cattle are clinically, physiologically, histopathologically, immunologically and reproductively normal.
Journal ArticleDOI
Sleep and sleep regulation in normal and prion protein-deficient mice.
TL;DR: It is suggested that PrP plays a role in promoting sleep continuity by comparing baseline recordings and the effects of sleep deprivation in PrP knockout mice and wild-type controls, which are the mice used for most gene targeting experiments and whose behavior is not well characterized.
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.