Doppel Protein Expression Correlates with Heme Oxygenase 1 and Nitric Oxide Synthase Induction

  • Boon-Seng Wong
  • Man-Sun Sy
  • David R. Brown


In the early 1990’s discrepant phenotypes were observed in four reported prion protein gene (Prnp)-ablated transgenic mouse lines (Prnp 0/0 ) (Bueler et al., 1992; Manson et al., 1994; Moore et al., 1995; Sakaguchi et al., 1996). Two of them, Ngsk and Rcm0 Prnp 0/0 mice (Moore et al., 1999; Sakaguchi et al., 1996), displayed normal early development but revealed progressive ataxia accompanied by the degeneration of cerebellar Purkinje cells at ~70 weeks of age. This phenotype was not observed in the other mouse lines, Zrch1 and Npu Prnp 0/0 mice (Bueler et al., 1992; Manson et al., 1994). Further investigation on the Rcm0 Prnp 0/0 mice lead to the discovery of a prion protein (PrP)-like protein named Doppel (Dpl) (Moore et al., 1999).


Nitric Oxide Prion Protein Heme Oxygenase Cerebellar Purkinje Cell Purkinje Cell Loss 
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Copyright information

© Springer Science+Business Media New York 2002

Authors and Affiliations

  • Boon-Seng Wong
    • 1
  • Man-Sun Sy
    • 1
  • David R. Brown
    • 2
  1. 1.Institute of PathologyCase Western Reserve UniversityClevelandUSA
  2. 2.Department of Biology and BiochemistryUniversity of BathBathUK

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