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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Cellular prion protein mediates impairment of synaptic plasticity by amyloid-β oligomers
TL;DR: The cellular prion protein (PrPC) is identified as an amyloid-β-oligomer receptor by expression cloning, and PrPC-specific pharmaceuticals may have therapeutic potential for Alzheimer’s disease.
Journal ArticleDOI
The cellular prion protein binds copper in vivo
David R. Brown,Kefeng Qin,Jochen Herms,Axel Madlung,Jean Manson,Robert Strome,Paul E. Fraser,T. P. A. Kruck,A von Bohlen,Walter J. Schulz-Schaeffer,Armin Giese,David Westaway,Hans A. Kretzschmar +12 more
TL;DR: Findings indicate that PrPC can exist in a Cu-metalloprotein form in vivo, and that its amino terminus contains the octapeptide PHGGGWGQ, which is among the best-preserved regions of mammalian PrPC.
Journal ArticleDOI
Prion protein biology.
TL;DR: This research was supported by grants from the National Institute of Aging and the National institute of Neurologic Diseases and Stroke of the National Institutes of Health, International Human Frontiers of Science Program, and American Health Assistance Foundation, as well as by gifts from the Sherman Fairchild Foundation, Keck Foundation, G. Mathers Foundation, Bernard Osher Foundation, and Centeon.
Journal ArticleDOI
Normal host prion protein necessary for scrapie-induced neurotoxicity
Sebastian Brandner,Stefan Isenmann,Alex Raeber,Marek Fischer,Andreas W. Sailer,Yasushi Kobayashi,Silvia Marino,Charles Weissmann,Adriano Aguzzi +8 more
TL;DR: In addition to being resistant to scrapie infection, brain tissue devoid of PrPc is not damaged by exogenous PrPSc, and even 16 months after inoculation no pathological changes were seen in PrP-deficient tissue, not even in the immediate vicinity of the grafts.
References
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Journal ArticleDOI
Linkage of the scrapie-associated fibril protein (PrP) gene and Sinc using congenic mice and restriction fragment length polymorphism analysis.
TL;DR: A cDNA copy of the hamster PrP mRNA is used to carry out restriction fragment length polymorphism (RFLP) analysis of different inbred mouse strains and the polymorphic XbaI site is mapped to the 3' flanking region of the PrP gene.
Journal ArticleDOI
Post-mortem immunodiagnosis of scrapie and bovine spongiform encephalopathy.
TL;DR: Two polyclonal antisera were raised in rabbits against the scrapie-associated fibril protein (PrP) prepared from sheep and mice which were terminally infected with experimental scrapie and may allow the identification of infected animals prior to the onset of clinical signs.
Journal ArticleDOI
An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease.
Frank Owen,Mark Poulter,T. Shah,John Collinge,R. Lofthouse,Harry F. Baker,Rosalind M. Ridley,John H. McVey,Timothy J. Crow +8 more
TL;DR: In a pedigree with Creutzfeldt-Jakob disease, a 144-bp insertion in the open reading frame of the prion protein (PrP) gene is identified and codes for 6 extra uninterrupted octapeptide repeats in addition to the 5 that are normally present in the N-terminal region of the protein.
Journal ArticleDOI
Diagnosis of Gerstmann-Sträussler syndrome in familial dementia with prion protein gene analysis.
John Collinge,Frank Owen,R. Lofthouse,T. Shah,A. E. Harding,Mark Poulter,A.M. Boughey,T. J. Crow +7 more
TL;DR: The polymerase chain reaction was used to screen DNA samples from 12 unrelated individuals with various familial dementias and ataxias for mutation in part of the prion protein (PrP) gene, an abnormality that occurs in individuals with the spongiform encephalopathies, Gerstmann-Sträussler syndrome and Creutzfeldt-Jakob disease.
Journal ArticleDOI
Different scrapie-associated fibril proteins (PrP) are encoded by lines of sheep selected for different alleles of the Sip gene.
TL;DR: By sequencing the protein coding region of the PrP gene in Cheviot sheep selected for differing Sip genotypes, it is found four PrP protein variants which differ at three positions: amino acid 112 (Ala/Val), amino acid 130 (Arg/His) and amino acid 147 ( Arg/Gln).