Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model. - Wikidata - wikimedia - TriplyDB

Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model.

Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model.

http://www.wikidata.org/entity/Q48417286

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Control of sleep and wakefulness Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice Fatal prion disease in a mouse model of genetic E200K Creutzfeldt-Jakob disease NMR Structure of the Human Prion Protein with the Pathological Q212P Mutation Reveals Unique Structural Features Expression of mutant or cytosolic PrP in transgenic mice and cells is not associated with endoplasmic reticulum stress or proteasome dysfunction Mutant PrP suppresses glutamatergic neurotransmission in cerebellar granule neurons by impairing membrane delivery of VGCC α(2)δ-1 Subunit Spontaneous generation of rapidly transmissible prions in transgenic mice expressing wild-type bank vole prion protein.Immunopurification of pathological prion protein aggregates Filamentous white matter prion protein deposition is a distinctive feature of multiple inherited prion diseases Mutant prion protein expression is associated with an alteration of the Rab GDP dissociation inhibitor alpha (GDI)/Rab11 pathway Mouse models for studying the formation and propagation of prions.Strain specific resistance to murine scrapie associated with a naturally occurring human prion protein polymorphism at residue 171.De novo prions.Prions.Review: contribution of transgenic models to understanding human prion disease.The genetic and molecular regulation of sleep: from fruit flies to humans.Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.Spongiform encephalopathy in transgenic mice expressing a point mutation in the β2-α2 loop of the prion protein.The elusive role of the prion protein and the mechanism of toxicity in prion disease.Ion channels induced by the prion protein: mediators of neurotoxicity.Cellular prion protein: from physiology to pathology.Quantifying prion disease penetrance using large population control cohorts PrP(ST), a soluble, protease resistant and truncated PrP form features in the pathogenesis of a genetic prion disease.Aggregated, wild-type prion protein causes neurological dysfunction and synaptic abnormalities.Prions: Beyond a Single Protein.Circadian clocks and neurodegenerative diseases: time to aggregate?A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP.Activation of the unfolded protein response and granulovacuolar degeneration are not common features of human prion pathology.Synaptic dysfunction in prion diseases: a trafficking problem?Selective vulnerability to neurodegenerative disease: the curious case of Prion Protein.Prion protein misfolding, strains, and neurotoxicity: an update from studies on Mammalian prions.Neuropathology of sleep disorders: a review.Mammalian sleep genetics.Genetic human prion disease modelled in PrP transgenic Drosophila.The Protein-disulfide Isomerase ERp57 Regulates the Steady-state Levels of the Prion Protein.Fatal Familial Insomnia: Clinical Aspects and Molecular Alterations.Experimental Models of Inherited PrP Prion Diseases.Prion protein facilitates uptake of zinc into neuronal cells.The hydrophobic core region governs mutant prion protein aggregation and intracellular retention.Insights into Mechanisms of Transmission and Pathogenesis from Transgenic Mouse Models of Prion Diseases.

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P2860

Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model.

http://www.wikidata.org/entity/Q48417286

description

2008 nî lūn-bûn

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2008年の論文

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2008年学术文章

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2008年学术文章

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2008年学术文章

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2008年学术文章

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2008年学术文章

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2008年学术文章

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2008年學術文章

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2008年學術文章

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Mutant prion protein expressio ...... s in a transgenic mouse model.

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Mutant prion protein expressio ...... s in a transgenic mouse model.

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Mutant prion protein expressio ...... s in a transgenic mouse model.

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Mutant prion protein expressio ...... s in a transgenic mouse model.

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Mutant prion protein expressio ...... s in a transgenic mouse model.

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J.NEURON.2008.09.008

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Mutant prion protein expressio ...... ns in a transgenic mouse model

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Alessandro Pincherle

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Anna Garofoli

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Assunta Senatore

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Claudia Balducci

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Fabio Fiordaliso

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Gabriella Marcon

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Gianluigi Forloni

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10.1016/J.NEURON.2008.09.008

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4

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Fabrizio Tagliavini

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2008-11-01T00:00:00Z

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23502000

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Prion protein family