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Prion propagation and toxicity in vivo occur in two distinct mechanistic phases
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α-cleavage of the prion protein occurs in a late compartment of the secretory pathway and is independent of lipid rafts
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Trans-Dominant Inhibition of Prion Propagation In Vitro Is Not Mediated by an Accessory Cofactor
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Soluble oligomers of the amyloid β-protein impair synaptic plasticity and behavior
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Effective Gene Therapy in a Mouse Model of Prion Diseases
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Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease
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The dominant-negative effect of the Q218K variant of the prion protein does not require protein X
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Toward molecular dissection of PrPC-PrPSc interactions
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Targeting cellular prion protein reverses early coanitive deficits and neurophysiological dysfunction in prion-infected mice
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Lethal recessive myelin toxicity of prion protein lacking its central domain
Frank Baumann et al.
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Neonatal lethality in transgenic mice expressing prion protein with a deletion of residues 105-125
Aimin Li et al.
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The C-terminal products of cellular prion protein processing, C1 and C2, exert distinct influence on p53-dependent staurosporine-induced caspase-3 activation
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The disintegrin ADAM9 indirectly contributes to the physiological processing of cellular prion by modulating ADAM10 activity
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The N-terminal cleavage of cellular prion protein in the human brain
I Laffont-Proust et al.
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Reactive oxygen species-mediated β-cleavage of the prion protein in the cellular response to oxidative stress
NT Watt et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
The AGAAAAGA palindrome in PrP is required to generate a productive PrPSc-PrPC complex that leads to prion propagation
EM Norstrom et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Dual mechanisms for shedding of the cellular prion protein
ET Parkin et al.
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Alpha- and beta- cleavages of the amino-terminus of the cellular prion protein
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Motif-grafted antibodies containing the replicative interface of cellular PrP are specific for PrPSc
G Moroncini et al.
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Doppel-induced Purkinje cell death is stoichiometrically abrogated by prion protein
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BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2004)
Calpain-dependent endoproteolytic cleavage of PrPSc modulates scrapie prion propagation
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JOURNAL OF BIOLOGICAL CHEMISTRY (2004)
Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis
G Mallucci et al.
SCIENCE (2003)
The mechanism of internalization of glycosylphosphatidylinositol-anchored prion protein
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Dominant-negative inhibition of prion replication in transgenic mice
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PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2002)
Endocytic intermediates involved with the intracellular trafficking of a fluorescent cellular prion protein
AC Magalhaes et al.
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Naturally secreted oligomers of amyloid β protein potently inhibit hippocampal long-term potentiation in vivo
DM Walsh et al.
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Doppel-induced cerebellar degeneration in transgenic mice
RC Moore et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2001)
Prion diseases: What is the neurotoxic molecule?
R Chiesa et al.
NEUROBIOLOGY OF DISEASE (2001)
Changing a single amino acid in the N-terminus of murine PrP alters TSE incubation time across three species barriers
RM Barron et al.
EMBO JOURNAL (2001)
Antibodies inhibit prion propagation and clear cell cultures of prion infectivity
D Peretz et al.
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Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody
M Enari et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2001)
Inhibition of interactions and interconversions of prion protein isoforms by peptide fragments from the C-terminal folded domain
M Horiuchi et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2001)
Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brain
D Rossi et al.
EMBO JOURNAL (2001)
Cleavage of the amino terminus of the prion protein by reactive oxygen species
HEM McMahon et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2001)
Phorbol ester-regulated cleavage of normal prion protein in HEK293 human cells and murine neurons
B Vincent et al.
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Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in PrP knockout mice
E Flechsig et al.
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