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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Book ChapterDOI
Transgenic studies of the influence of the PrP structure on TSE diseases.
Emmanuel A. Asante,John Collinge +1 more
TL;DR: Transgenic technology has made an invaluable contribution to acquisition of new knowledge in prion biology, particularly in structural studies, which has recently attracted remarkable attention.
Journal ArticleDOI
Loss of functional prion protein: a role in prion disorders?
TL;DR: Experimental evidence suggests that the loss of functional PrP is not the instigating factor in prion disorders, and this work aims to clarify the role of PrP in these disorders.
Journal ArticleDOI
What Is Our Current Understanding of PrPSc-Associated Neurotoxicity and Its Molecular Underpinnings?
Daniel Hughes,Mark Halliday +1 more
TL;DR: The diseases caused by PrPSc toxicity, the nature of the toxicity ofPrPSc, and the current understanding of the downstream toxic signaling events triggered by the presence of PrP sc are discussed.
Book ChapterDOI
The role of the immune system in prion infection.
TL;DR: This chapter describes the interactions between prions and the host's immune system and describes how prion disease pathogenesis and susceptibility may be influenced by inflammation, co-infection with other pathogens, and aging.
Journal ArticleDOI
Loss of prion protein is associated with the development of insulin resistance and obesity.
Giovanna Brito,Fernada C S Lupinacci,Flavio H. Beraldo,Tiago G. Santos,Martín Roffé,Marilene H. Lopes,Vladmir Cláudio Cordeiro de Lima,Vilma R. Martins,Glaucia N. M. Hajj +8 more
TL;DR: The results suggest that PrPC reflects susceptibility to the development of insulin resistance and metabolic syndrome.
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.