title: The PrP-like proteins Shadoo and Doppel.
abstract: An almost unique place within protein databases, twenty-five years of study has underscored the enigmatically subtle role of PrP(C) 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 PrP(C) 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 PrP(C) itself. These molecules may be simpler - and more "understandable" - entities that can be pursued in parallel to PrP(C), 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.
authors: Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, AB, Canada. david.westaway@ualberta.ca
authors: David
authors: D
authors: Westaway
authors: Nathalie
authors: N
authors: Daude
authors: Serene
authors: S
authors: Wohlgemuth
authors: Paul
authors: P
authors: Harrison
mesh_terms: D000483:Alleles; D000595:Amino Acid Sequence; D000818:Animals; D001703:Biophysics / Q000379:methods; D002462:Cell Membrane / Q000378:metabolism; D058851:GPI-Linked Proteins / Q000378:metabolism / Q000502:physiology; D006801:Humans; D008954:Models, Biological; D008969:Molecular Sequence Data; D009419:Nerve Tissue Proteins / Q000378:metabolism / Q000502:physiology*; D009474:Neurons / Q000473:pathology; D011110:Polymorphism, Genetic; D011328:Prions / Q000737:chemistry* / Q000378:metabolism / Q000502:physiology; D017434:Protein Structure, Tertiary; D017386:Sequence Homology, Amino Acid
chemical_list: D058851:GPI-Linked Proteins; D009419:Nerve Tissue Proteins; C501125:PRND protein, human; D011328:Prions; C478971:SPRN protein, human