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Gerstmann-Straussler-Scheinker Disease
Known as:
Gerstmann Straussler Scheinker Syndrome
, Gerstmann-Straussler-Scheinker Syndrome
, Gerstmann-Straussler Inherited Spongiform Encephalopathy
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An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal…
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National Institutes of Health
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Related topics
Related topics
33 relations
Aggressive behavior
Apraxias
Ataxia, Truncal
Autosomal dominant inheritance
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Broader (2)
Prion Diseases
Slow 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
L. Fioriti
,
N. Angeretti
,
+8 authors
G. Forloni
Journal of Neuroscience
2007
Corpus ID: 6870943
Amyloid fibrils in Gerstmann–Sträussler–Scheinker (GSS) disease are composed of a fragment of the prion protein (PrP), the N and…
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Review
2003
Review
2003
Hereditary prion protein amyloidoses.
B. Ghetti
,
F. Tagliavini
,
Masaki Takao
,
O. Bugiani
,
P. Piccardo
Clinics in Laboratory Medicine
2003
Corpus ID: 41103314
Highly Cited
2001
Highly Cited
2001
Flexibility of the murine prion protein and its Asp178Asn mutant investigated by molecular dynamics simulations.
Jörg Gsponer
,
Philippe Ferrara
,
A. Caflisch
Journal of Molecular Graphics and Modelling
2001
Corpus ID: 608177
1999
1999
Mutations in the glucose‐6‐phosphate transporter (G6PT) gene in patients with glycogen storage diseases type 1b and 1c
L. Galli
,
A. Orrico
,
+8 authors
V. Sorrentino
FEBS Letters
1999
Corpus ID: 41229244
1999
1999
Prion protein glycotype analysis in familial and sporadic Creutzfeldt-Jakob disease patients
F. Cardone
,
Q. G. Liu
,
+6 authors
M. Pocchiari
Brain Research Bulletin
1999
Corpus ID: 41154111
Review
1996
Review
1996
Blood proteins from transgenic animal bioreactors.
H. Luboń
,
R. Paleyanda
,
W. Velander
,
W. Drohan
Transfusion Medicine Reviews
1996
Corpus ID: 37310132
Highly Cited
1993
Highly Cited
1993
ßPP Participates in PrP-Amyloid Plaques of Gerstmann-Sträussler-Scheinker Disease, Indiana Kindred
O. Bugiani
,
G. Giaccone
,
+5 authors
Bernardino Ghett
Journal of Neuropathology and Experimental…
1993
Corpus ID: 11045016
Gerstmann-sträussler-Scheinker disease in the Indiana kindred is pathologically characterized by deposits of PrP-amyloid…
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1993
1993
An Israeli family with Gerstmann‐Straussler‐Scheinker disease manifesting the codon 102 mutation in the prion protein gene
Y. Goldhammer
,
R. Gabizon
,
Z. Meiner
,
M. Sadeh
Neurology
1993
Corpus ID: 8018166
We report the first family among the Jewish population in Israel with Gerstmann-Straussler-Scheinker disease. A proline-for…
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Review
1991
Review
1991
The human spongiform encephalopathies: kuru, Creutzfeldt-Jakob disease, and the Gerstmann-Sträussler-Scheinker syndrome.
P. Brown
,
D. Gajdusek
Current Topics in Microbiology and Immunology
1991
Corpus ID: 29024708
Kuru and the transmissible virus dementias Creutzfeldt-Jakob disease (CJD) and Gerstmann-Straussler-Scheinker syndrome (GSS…
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1990
1990
Spongiform encephalopathy transmitted experimentally from Creutzfeldt-Jakob and familial Gerstmann-Sträussler-Scheinker diseases.
H. Baker
,
L. Duchen
,
J. Jacobs
,
R. Ridley
Brain : a journal of neurology
1990
Corpus ID: 42803162
A comparison was made of the effects of experimental intracerebral inoculation into marmosets of brain homogenates from a case of…
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