Journal ArticleDOI
129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal.
Reads0
Chats0
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
More filters
Journal ArticleDOI
The association between PrP and infectivity in scrapie and BSE infected mouse brain
R. A. Somerville,A. J. Dunn +1 more
TL;DR: It is concluded that at least some PrPSc in the brain need not be associated directly with infectious agent but is deposited in brain solely as a pathological product of infection.
Journal ArticleDOI
Prion gene paralogs are dispensable for early zebrafish development and have nonadditive roles in seizure susceptibility.
TL;DR: Prion mutant fish lack the overt phenotypes previously predicted, and instead they have subtle phenotypes similar to mammals, consistent with ancient functions of prion proteins in neurodevelopment and modulation of neural activity.
Journal ArticleDOI
PrP effects clarified
TL;DR: It is not possible to attribute the observed phenotype to ablation of PrP because correction of the phenotype through introduction of a PrP-expressing cDNA has not been carried out.
Book ChapterDOI
Transgenic mouse models of prion diseases.
TL;DR: This paper reviewed recent advances in the understanding of prion biology that derive from this powerful and informative approach, and pointed out that prions represent a new biological paradigm of protein-mediated information transfer.
Journal ArticleDOI
Serum withdrawal-induced apoptosis in ZrchI prion protein (PrP) gene-deficient neuronal cell line is suppressed by PrP, independent of Doppel.
Takuya Nishimura,Akikazu Sakudo,Akikazu Sakudo,Yoriko Hashiyama,Akiko Yachi,Keiichi Saeki,Yoshitsugu Matsumoto,Masaharu Ogawa,Suehiro Sakaguchi,Suehiro Sakaguchi,Shigeyoshi Itohara,Takashi Onodera +11 more
TL;DR: Results indicate that Dpl production did not affect anti‐apoptotic and anti‐oxidative functions of PrP, suggesting that PrPC may be directly correlated with protection against oxidative stress.
References
More filters
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.