The original Gerstmann-Sträussler-Scheinker family of Austria: divergent clinicopathological phenotypes but constant PrP genotype (Q47199003)

11 January 2018

title

The original Gerstmann-Sträussler-Scheinker family of Austria: divergent clinicopathological phenotypes but constant PrP genotype (English)

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11 January 2018

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8

Liberski PP

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author name string

Hainfellner JA

1

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Brantner-Inthaler S

2

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Cervenáková L

3

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Brown P

4

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Kitamoto T

5

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Tateishi J

6

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Diringer H

7

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Regele H

9

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Feucht M

10

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publication date

1 July 1995

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11 January 2018

published in

Brain Pathology

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volume

5

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page(s)

201-211

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issue

3

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Spongiform encephalopathy transmitted experimentally from Creutzfeldt-Jakob and familial Gerstmann-Sträussler-Scheinker diseases.

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Microglia is a component of the prion protein amyloid plaque in the Gerstmann-Sträussler-Scheinker syndrome.

21 January 2018

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Prion dementia without characteristic pathology

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Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease

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Ballooned neurons in select neurodegenerative diseases contain phosphorylated neurofilament epitopes.

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Pro----leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Sträussler syndrome

21 January 2018

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Neurofibrillary tangles of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease share antigenic determinants with those of Alzheimer disease.

21 January 2018

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An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann-Sträussler-Scheinker family

21 January 2018

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Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism

21 January 2018

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Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome

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Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles

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Inherited human prion diseases

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A prion protein variant in a family with the telencephalic form of Gerstmann-Straussler-Scheinker syndrome

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Proteinase-resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity

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An amber mutation of prion protein in Gerstmann-Sträussler syndrome with mutant PrP plaques

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Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Sträussler syndrome

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Molecular cloning of a human prion protein cDNA

21 January 2018

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Gerstmann-Sträussler-Scheinker's disease

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Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies

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Dystrophic neurites around amyloid plaques of human patients with Gerstmann-Sträussler-Scheinker disease contain ubiquitinated inclusions

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Familial dementia with PrP-positive amyloid plaques: a variant of Gerstmann-Sträussler syndrome

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Characterisation of antisera raised against species-specific peptide sequences from scrapie-associated fibril protein and their application for post-mortem immunodiagnosis of spongiform encephalopathies.

21 January 2018

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Homozygous prion protein genotype predisposes to sporadic Creutzfeldt–Jakob disease

21 January 2018

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Molecular biology of prion diseases

21 January 2018

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10.1111/J.1750-3639.1995.TB00596.X

21 January 2018

Identifiers

DOI

1 reference

11 January 2018

release_xvcjrcsbofaqxp2liym67ul3eu

Fatcat ID

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/xvcjrcsbofaqxp2liym67ul3eu

mapped directly with Wikidata item

24 November 2022

based on heuristic

PubMed ID

8520719

1 reference

11 January 2018