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
Citations
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Prion Protein Interacts with BACE1 Protein and Differentially Regulates Its Activity toward Wild Type and Swedish Mutant
Heledd H. Griffiths,Isobel J. Whitehouse,Herbert Baybutt,Debbie Brown,Katherine A. B. Kellett,Carolyn D. Jackson,Anthony J. Turner,Pedro Piccardo,Jean Manson,Nigel M. Hooper +9 more
TL;DR: In this article, the cellular prion protein (PrP C ) inhibited the action of BACE1 toward human wild type amyloid precursor protein (APPWT) in cellular models and that the levels of endogenous murine A were significantly increased in PrP C -null mouse brain.
Journal ArticleDOI
Generation of a new infectious recombinant prion: a model to understand Gerstmann–Sträussler–Scheinker syndrome
Saioa R. Elezgarai,Natalia Fernández-Borges,Hasier Eraña,Alejandro M. Sevillano,Jorge M. Charco,Chafik Harrathi,Paula Saá,David Gil,Qingzhong Kong,Jesús R. Requena,Olivier Andreoletti,Joaquín Castilla +11 more
TL;DR: This study proves the possibility to generate recombinant versions of other human prion diseases that could provide a further understanding on the molecular features of these devastating disorders.
Journal ArticleDOI
Bovine Spongiform Encephalopathy in Japan: History and Recent Studies on Oxidative Stress in Prion Diseases
TL;DR: Present results support the hypothesis that ‘loss‐of‐function’ of PrPC decreases resistance to oxidative stress, and ‘gain‐of-function' of PrPSc increases oxidative stress.
Book ChapterDOI
Prions: from neurografts to neuroinvasion.
TL;DR: The state of knowledge on the routes of neuroinvasion used by the infectious agent in order to gain access to the central nervous system upon entry into extracerebral sites is reviewed.
Journal ArticleDOI
Hematological shift in goat kids naturally devoid of prion protein.
Malin Rokseth Reiten,Maren Kolltveit Bakkebø,Hege Brun-Hansen,Anna Lewandowska-Sabat,Ingrid Olsaker,Michael A. Tranulis,Arild Espenes,Preben Boysen +7 more
TL;DR: The data suggest that PrPC has a role in bone marrow physiology and warrant further studies of PrPC in erythroid and immune cell progenitors as well as differentiated effector cells also under stressful conditions.
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.