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Symbol PRNP contributors: mct/shn - updated : 26-12-2013
HGNC name prion protein
HGNC id 9449
  • 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
  • ablation of the prion protein PrP(C) triggers a chronic demyelinating polyneuropathy in four independently targeted mouse strains
  • absence of PrPC in a transgenic Alzheimer mouse rescues deficits in spatial learning and memory
  • Knockdown PRNP homolog in the zebrafish leads to a dose-dependent phenotype characterized by systemic morphological defects, reduced cell adhesion and CNS cell death