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| mice expressing DeltaPrP or Dpl suffer from widespread leukoencephalopathy  | |
mouse expressing the PrP(DeltaCD), a PrP variant lacking 40 central residues, displays a rapidly progressive, lethal phenotype with extensive central and peripheral myelin degeneration |
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ablation of the prion protein PrP(C) triggers a chronic demyelinating polyneuropathy in four independently targeted mouse strains |
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absence of PrPC in a transgenic Alzheimer mouse rescues deficits in spatial learning and memory |
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Knockdown PRNP homolog in the zebrafish leads to a dose-dependent phenotype characterized by systemic morphological defects, reduced cell adhesion and CNS cell death |