Prion disease modelled in Drosophila
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Authors
Smith, Andrew
Fleck, Oliver
Spiropoulos, John
Andreoletti, Olivier
Journal Title
Cell and Tissue Research
ISSN
0302-766X
Publisher
Springer
Type
Article
This Version
AM
Metadata
Show full item recordCitation
Bujdoso, R., Smith, A., Fleck, O., Spiropoulos, J., Andreoletti, O., & Thackray, A. Prion disease modelled in Drosophila. Cell and Tissue Research https://doi.org/10.17863/CAM.80400
Abstract
Prion diseases are fatal neurodegenerative conditions of humans and various vertebrate species that are transmissible between individuals of the same or different species. A novel infectious moiety referred to as a prion is considered responsible for transmission of these conditions. Prion replication is believed to be the cause of the neurotoxicity that arises during prion disease pathogenesis. The prion hypothesis predicts that the transmissible prion agent consists of PrPSc, which is comprised of aggregated misfolded conformers of the normal host protein PrPC. It is important to understand the biology of transmissible prions and to identify genetic modifiers of prion-induced neurotoxicity. This information will underpin the development of therapeutic and control strategies for human and animal prion diseases. The most reliable method to detect prion infectivity is by in-vivo transmission in a suitable experimental host, which to date have been mammalian species. Current prion bioassays are slow, cumbersome and relatively insensitive to low titres of prion infectivity, and do not lend themselves to rapid genetic analysis of prion disease. Here we provide an over-view of our novel studies that have led to the establishment of Drosophila melanogaster, a genetically well-defined invertebrate host, as a sensitive, versatile and economically viable animal model for the detection of mammalian prion infectivity and genetic modifiers of prion-induced toxicity.
Sponsorship
National Centre for the Replacement Refinement and Reduction of Animals in Research (NC/K000462/1)
National Centre for the Replacement Refinement and Reduction of Animals in Research (NC/R00093X/1)
BBSRC (BB/T00343X/1)
Embargo Lift Date
2025-01-27
Identifiers
This record's DOI: https://doi.org/10.17863/CAM.80400
This record's URL: https://www.repository.cam.ac.uk/handle/1810/332976
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