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- W2012109320 abstract "The downstream prion-like protein (doppel, or Dpl) is a paralog of the cellular prion protein, PrP C . The two proteins have ≈25% sequence identity, but seem to have distinct physiologic roles. Unlike PrP C , Dpl does not support prion replication; instead, overexpression of Dpl in the brain seems to cause a completely different neurodegenerative disease. We report the solution structure of a fragment of recombinant mouse Dpl (residues 26–157) containing a globular domain with three helices and a small amount of β-structure. Overall, the topology of Dpl is very similar to that of PrP C . Significant differences include a marked kink in one of the helices in Dpl, and a different orientation of the two short β-strands. Although the two proteins most likely arose through duplication of a single ancestral gene, the relationship is now so distant that only the structures retain similarity; the functions have diversified along with the sequence." @default.
- W2012109320 created "2016-06-24" @default.
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- W2012109320 date "2001-02-27" @default.
- W2012109320 modified "2023-10-13" @default.
- W2012109320 title "Two different neurodegenerative diseases caused by proteins with similar structures" @default.
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- W2012109320 doi "https://doi.org/10.1073/pnas.051627998" @default.
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