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Gerstmann-Straussler-Scheinker Disease

Known as: Gerstmann Straussler Scheinker Syndrome, Gerstmann-Straussler-Scheinker Syndrome, Gerstmann-Straussler Inherited Spongiform Encephalopathy 
An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal… 
National Institutes of Health

Related topics

Related topics

33 relations
Aggressive behaviorApraxiasAtaxia, TruncalAutosomal dominant inheritance

Broader (2)

Prion DiseasesSlow Virus Diseases

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2007
2007
Neurotoxic and Gliotrophic Activity of a Synthetic Peptide Homologous to Gerstmann–Sträussler–Scheinker Disease Amyloid Protein
Amyloid fibrils in Gerstmann–Sträussler–Scheinker (GSS) disease are composed of a fragment of the prion protein (PrP), the N and… 
Review
2003
Review
2003
Hereditary prion protein amyloidoses.
Highly Cited
2001
Highly Cited
2001
Flexibility of the murine prion protein and its Asp178Asn mutant investigated by molecular dynamics simulations.
1999
1999
Mutations in the glucose‐6‐phosphate transporter (G6PT) gene in patients with glycogen storage diseases type 1b and 1c
1999
1999
Prion protein glycotype analysis in familial and sporadic Creutzfeldt-Jakob disease patients
Review
1996
Review
1996
Blood proteins from transgenic animal bioreactors.
Highly Cited
1993
Highly Cited
1993
ßPP Participates in PrP-Amyloid Plaques of Gerstmann-Sträussler-Scheinker Disease, Indiana Kindred
Gerstmann-sträussler-Scheinker disease in the Indiana kindred is pathologically characterized by deposits of PrP-amyloid… 
1993
1993
An Israeli family with Gerstmann‐Straussler‐Scheinker disease manifesting the codon 102 mutation in the prion protein gene
We report the first family among the Jewish population in Israel with Gerstmann-Straussler-Scheinker disease. A proline-for… 
Review
1991
Review
1991
The human spongiform encephalopathies: kuru, Creutzfeldt-Jakob disease, and the Gerstmann-Sträussler-Scheinker syndrome.
Kuru and the transmissible virus dementias Creutzfeldt-Jakob disease (CJD) and Gerstmann-Straussler-Scheinker syndrome (GSS… 
1990
1990
Spongiform encephalopathy transmitted experimentally from Creutzfeldt-Jakob and familial Gerstmann-Sträussler-Scheinker diseases.
A comparison was made of the effects of experimental intracerebral inoculation into marmosets of brain homogenates from a case of… 
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