Prion Proteins pp 225-256 | Cite as

The PrP-Like Proteins Shadoo and Doppel

  • David Westaway
  • Nathalie Daude
  • Serene Wohlgemuth
  • Paul Harrison
Part of the Topics in Current Chemistry book series (TOPCURRCHEM, volume 305)

Abstract

An almost unique place within protein databases, twenty-five years of study has underscored the enigmatically subtle role of PrPC in normal cell biology. It seems that PrP has evolved (and survived) to perform a function that does not have a precedent amongst transmembrane cell-surface proteins, perhaps representing a new type of plasma membrane ecosystem. In a context where we await a clarifying insight to unify a panoply of PrPC data into logical molecular framework, the GPI-anchored N-glycosylated Doppel and Sho proteins are tantalizing in that they correspond roughly to the front and back halves of PrPC itself. These molecules may be simpler – and more “understandable” – entities that can be pursued in parallel to PrPC, and could open up the biology of mammalian prion proteins from fresh directions. Dpl has a profound role in successful gametogenesis that warrants close scrutiny and a case for deeper study can be made for Sho, a recently discovered CNS-expressed protein with many parallels to established facets of PrP biochemistry. In an aerial view of biomedical research, Sho and Dpl can be considered as adjacent islands in a prion protein archipelago. As such, the coming years of molecular exploration should be extremely interesting.

Graphical Abstract

Keywords

Creutzfeldt–Jakob Disease Doppel Neuroprotection Prion Shadoo 

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Copyright information

© Springer-Verlag Berlin Heidelberg 2011

Authors and Affiliations

  • David Westaway
    • 1
  • Nathalie Daude
    • 1
  • Serene Wohlgemuth
    • 1
  • Paul Harrison
    • 2
  1. 1.Centre for Prions and Protein Folding DiseasesUniversity of AlbertaEdmontonCanada
  2. 2.Department of BiologyMcGill UniversityMontrealCanada

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