Abstract
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).
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Wong, BS., Sy, MS., Brown, D.R. (2002). Doppel Protein Expression Correlates with Heme Oxygenase 1 and Nitric Oxide Synthase Induction. In: Abraham, N.G. (eds) Heme Oxygenase in Biology and Medicine. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-0741-3_37
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DOI: https://doi.org/10.1007/978-1-4615-0741-3_37
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