Chapter

Prion Proteins

Volume 305 of the series Topics in Current Chemistry pp 225-256

Date:

The PrP-Like Proteins Shadoo and Doppel

  • David WestawayAffiliated withCentre for Prions and Protein Folding Diseases, University of Alberta Email author 
  • , Nathalie DaudeAffiliated withCentre for Prions and Protein Folding Diseases, University of Alberta
  • , Serene WohlgemuthAffiliated withCentre for Prions and Protein Folding Diseases, University of Alberta
  • , Paul HarrisonAffiliated withDepartment of Biology, McGill University

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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

https://static-content.springer.com/image/chp%3A10.1007%2F128_2011_190/MediaObjects/209549_1_En_190_Figa_HTML.gif

Keywords

Creutzfeldt–Jakob Disease Doppel Neuroprotection Prion Shadoo