ArticleHuman molecular genetics2010
A Drosophila model of GSS syndrome suggests defects in active zones are responsible for pathogenesis of GSS syndrome.
Article in Human molecular genetics, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 19 citations in OpenAlex.
- Genetic modulation of CWD prion propagation in cervid PrP Drosophila.The Biochemical journal · 2023Article
- Prion disease modelled in Drosophila.Cell and tissue research · 2023Review
- The Prion Protein Regulates Synaptic Transmission by Controlling the Expression of Proteins Key to Synaptic Vesicle Recycling and Exocytosis.Molecular neurobiology · 2019Article
- Mammalian prion propagation in PrP transgenic Drosophila.Brain : a journal of neurology · 2018Article
- Genetic human prion disease modelled in PrP transgenicThe Biochemical journal · 2017Article
- Drosophila models of prionopathies: insight into prion protein function, transmission, and neurotoxicity.Current opinion in genetics & development · 2017Review
- Prion protein facilitates synaptic vesicle release by enhancing release probability.Human molecular genetics · 2014Article
- Methods to assay Drosophila behavior.Journal of visualized experiments : JoVE · 2012Article
- Molecular mechanism of active zone organization at vertebrate neuromuscular junctions.Molecular neurobiology · 2012Review
- Presynaptic Active Zone Density during Development and Synaptic Plasticity.Frontiers in molecular neuroscience · 2012Article
- Drosophila models of proteinopathies: the little fly that could.Current pharmaceutical design · 2012Review
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Authors and funding
9 authors at 1 institution in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
We have established a Drosophila model of Gerstmann-Sträussler-Scheinker (GSS) syndrome by expressing mouse prion protein (PrP) having leucine substitution at residue 101 (MoPrP(P101L)). Flies expressing MoPrP(P101L), but not wild-type MoPrP (MoPrP(3F4)), showed severe defects in climbing ability and early death. Expressed MoPrP(P101L) in Drosophila was differentially glycosylated, localized at the synaptic terminals and mainly present as deposits in adult brains. We found that behavioral defects and early death of MoPrP(P101L) flies were not due to Caspase 3-dependent programmed cell death signaling. In addition, we found that Type 1 glutamatergic synaptic boutons in larval neuromuscular junctions of MoPrP(P101L) flies showed significantly increased numbers of satellite synaptic boutons. Furthermore, the amount of Bruchpilot and Discs large in MoPrP(P101L) flies was significantly reduced. Brains from scrapie-infected mice showed significantly decreased ELKS, an active zone matrix marker compared with those of age-matched control mice. Thus, altered active zone structures at the molecular level may be involved in the pathogenesis of GSS syndrome in Drosophila and scrapie-infected mice.
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