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 New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP.

The Journal of neuroscience : the official journal of the Society for Neuroscience - 12 Aug 2009

Yang Wenbin, Cook Julie, Rassbach Benjamin, Lemus Azucena, DeArmond Stephen J, Mastrianni James A

Abstract excerpt

Gerstmann-Sträussler-Scheinker syndrome (GSS) is a genetic prion disease typified clinically by the development of progressive ataxia and dementia, and histopathologically by the presence of prion protein (PrP) amyloid plaques in the CNS, especially within the cerebellum. Several mutations of the PrP gene (PRNP) are associated with GSS, but only the P102L mutation has been convincingly modeled in transgenic (Tg)...

Read the complete abstract on PubMed

Topics

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

Share to Ataxia
Share to Benzothiazoles

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Share to Creutzfeldt-Jakob Syndrome

Share to Gerstmann-Straussler-Scheinker Disease

Share to Prion Proteins

Share to PrPC Proteins

Share to PrPSc Proteins

Share to Thiazoles

Source

Open in PubMed Open full text

Identifiers

PMID: 19675240
DOI: 10.1523/JNEUROSCI.2542-09.2009
PMCID: PMC2749997

Genes

GSS
PRNP

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP.PMID 19675240Read ↗

A transgenic model of a familial prion disease.Semantic proximity Read ↗

A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles.Semantic proximity Read ↗

Neuropathology of Gerstmann-Sträussler-Scheinker disease.Semantic proximity Read ↗

Neurofibrillary tangles in Gerstmann-Sträussler-Scheinker syndrome with the A117V prion gene mutation.Semantic proximity Read ↗

Description of the first Spanish case of Gerstmann-Sträussler-Scheinker disease with A117V variant: clinical, histopathological and biochemical characterization.Semantic proximity Read ↗

Gerstmann-Sträussler-Scheinker disease and the French-Alsatian A117V variant.Semantic proximity Read ↗

The original Gerstmann-Sträussler-Scheinker family of Austria: divergent clinicopathological phenotypes but constant PrP genotype.Semantic proximity Read ↗

Spontaneous neurodegeneration in transgenic mice with mutant prion protein.Semantic proximity Read ↗

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

Transmissibility of Gerstmann-Sträussler-Scheinker syndrome in rodent models: New insights into the molecular underpinnings of prion infectivity.Semantic proximity Read ↗

Genetic and pathological features encipher the phenotypic heterogeneity of Gerstmann-Sträussler-Scheinker disease.Semantic proximity Read ↗

Immunodetection of disease-associated mutant PrP, which accelerates disease in GSS transgenic mice.Semantic proximity Read ↗

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