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Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein
PrPc is a host protein anchored to the outer surface of neurons and to a lesser extent of lymphocytes and other cells. The transmissible agent (prion) responsible for scrapie is believed to be aExpand
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Mice devoid of PrP are resistant to scrapie
S.B. Prusiner proposed that the infectious agent of scraple, the prion, is PrPSc, a modified form of the normal host protein PrPC. Prn-p0/0 mice devoid of PrPC showed normal development and behavior.Expand
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Prion protein (PrP) with amino‐proximal deletions restoring susceptibility of PrP knockout mice to scrapie.
The ‘protein only’ hypothesis postulates that the prion, the agent causing transmissible spongiform encephalopathies, is PrP(Sc), an isoform of the host protein PrP(C). Protease treatment of prionExpand
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Expression of Amino-Terminally Truncated PrP in the Mouse Leading to Ataxia and Specific Cerebellar Lesions
The physiological role of prion protein (PrP) remains unknown. Mice devoid of PrP develop normally but are resistant to scrapie; introduction of a PrP transgene restores susceptibility to theExpand
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Deficient signaling in mice devoid of double‐stranded RNA‐dependent protein kinase.
Double‐stranded RNA‐dependent protein kinase (PKR) has been implicated in interferon (IFN) induction, antiviral response and tumor suppression. We have generated mice devoid of functional PKR (Pkr%).Expand
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A quantitative, highly sensitive cell-based infectivity assay for mouse scrapie prions
Prions are usually quantified by bioassays based on intracerebral inoculation of mice that are slow, imprecise, and costly. We have isolated neuroblastoma N2a sublines highly susceptible to mouseExpand
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A rapid, sensitive, and specific method for the determination of protein in dilute solution.
Abstract A protein assay is described in which the sample is precipitated with trichloroacetic acid in the presence of sodium dodecylsulfate, filtered off on a Millipore membrane and stained withExpand
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A cellular gene encodes scrapie PrP 27-30 protein
A clone encoding PrP 27-30, the major protein in purified preparations of scrapie agent, was selected from a scrapie-infected hamster brain cDNA library by oligonucleotide probes corresponding to theExpand
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Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody
Exposure of susceptible neuroblastoma N2a cells to mouse scrapie prions leads to infection, as evidenced by the continued presence of the scrapie form of the prion protein (PrPSc) and infectivityExpand
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Normal host prion protein necessary for scrapie-induced neurotoxicity
ACCUMULATION of the prion protein PrPSc, a pathological and protease-resistant isoform of the normal host protein PrPc, is a feature of prion disease such as scrapie1,2. It is still unknown whetherExpand
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