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Citation
Fernandez-Funez, P., Zhang, Y., Casas-Tinto, S., Xiao, X., Zou, W.Q., Rincon-Limas, D.E. (2010). Sequence-dependent prion protein misfolding and neurotoxicity.  J. Biol. Chem. 285(47): 36897--36908.
FlyBase ID
FBrf0214139
Publication Type
Research paper
Abstract

Prion diseases are neurodegenerative disorders caused by misfolding of the normal prion protein (PrP) into a pathogenic "scrapie" conformation. To better understand the cellular and molecular mechanisms that govern the conformational changes (conversion) of PrP, we compared the dynamics of PrP from mammals susceptible (hamster and mouse) and resistant (rabbit) to prion diseases in transgenic flies. We recently showed that hamster PrP induces spongiform degeneration and accumulates into highly aggregated, scrapie-like conformers in transgenic flies. We show now that rabbit PrP does not induce spongiform degeneration and does not convert into scrapie-like conformers. Surprisingly, mouse PrP induces weak neurodegeneration and accumulates small amounts of scrapie-like conformers. Thus, the expression of three highly conserved mammalian prion proteins in transgenic flies uncovered prominent differences in their conformational dynamics. How these properties are encoded in the amino acid sequence remains to be elucidated.

PubMed ID
PubMed Central ID
PMC2978619 (PMC) (EuropePMC)
Related Publication(s)
Note

Pulling rabbits to reveal the secrets of the prion protein.
Fernandez-Funez et al., 2011, Commun. Integr. Biol. 4(3): 262--266 [FBrf0250079]

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Secondary IDs
    Language of Publication
    English
    Additional Languages of Abstract
    Parent Publication
    Publication Type
    Journal
    Abbreviation
    J. Biol. Chem.
    Title
    Journal of Biological Chemistry
    Publication Year
    1905-
    ISBN/ISSN
    0021-9258
    Data From Reference