Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

A Drosophila model of GSS syndrome suggests defects in active zones are responsible for pathogenesis of GSS syndrome

9 Sept 2010

Abstract excerpt

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...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Amyotrophic Lateral Sclerosis Research
Share to Prion Diseases and Protein Misfolding

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Source

Identifiers

PMID: 20829230
DOI: 10.1093/hmg/ddq379

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

A Drosophila model of GSS syndrome suggests defects in active zones are responsible for pathogenesis of GSS syndromePMID 20829230Read ↗

Drosophila models of prionopathies: insight into prion protein function, transmission, and neurotoxicity.Semantic proximity Read ↗

Reversible symptoms and clearance of mutant prion protein in an inducible model of a genetic prion disease in Drosophila melanogaster.Semantic proximity Read ↗

Fragile X premutation rCGG repeats impair synaptic growth and synaptic transmission at Drosophila larval neuromuscular junction.Semantic proximity Read ↗

Studies of neurodegenerative diseases using Drosophila and the development of novel approaches for their analysisSemantic proximity Read ↗

A candidate loss-of-function variant in SGIP1 causes synaptic dysfunction and recessive parkinsonism.Semantic proximity Read ↗

A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP.Semantic proximity Read ↗

Characterisation and prion transmission study in mice with genetic reduction of sporadic Creutzfeldt-Jakob Disease risk gene <i>Stx6</i>Semantic proximity Read ↗

New Drosophila models to uncover the intrinsic and extrinsic factors mediating the toxicity of the human prion proteinSemantic proximity Read ↗

Abnormal synaptic architecture in iPSC-derived neurons from a multi-generational family with genetic Creutzfeldt-Jakob disease.Semantic proximity Read ↗

Experimental Models of Inherited PrP Prion Diseases.Semantic proximity Read ↗

Characterisation and prion transmission study in mice with genetic reduction of sporadic Creutzfeldt-Jakob disease risk gene Stx6Semantic proximity Read ↗

Transcriptional signature of prion-induced neurotoxicity in a Drosophila model of transmissible mammalian prion disease.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.