(Q35122324)

6 August 2017

1 reference

title

Hereditary prion protein amyloidoses (English)

1 reference

6 August 2017

0 references

1

Bernardino Ghetti

1 reference

6 August 2017

2

Fabrizio Tagliavini

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A12733425

30 December 2023

author name string

Orso Bugiani

4

1 reference

6 August 2017

Pedro Piccardo

5

1 reference

6 August 2017

Bernardino Ghetti

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A12733425

30 December 2023

M. Takao

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A12733425

30 December 2023

language of work or name

English

1 reference

National Center for Biotechnology Information

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esummary.fcgi?db=pubmed&retmode=json&id=12733425

30 December 2023

publication date

1 March 2003

1 reference

full work available at URL

6 August 2017

https://api.elsevier.com/content/article/PII:S0272271202000641?httpAccept=text/xml

1 reference

https://api.crossref.org/works/10.1016/S0272-2712(02)00064-1

https://api.elsevier.com/content/article/PII:S0272271202000641?httpAccept=text/plain

30 December 2023

1 reference

https://api.crossref.org/works/10.1016/S0272-2712(02)00064-1

Clinics in laboratory medicine

30 December 2023

published in

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6 August 2017

volume

23

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6 August 2017

issue

1

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6 August 2017

page(s)

65-85, viii

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Prion protein amyloidosis

6 August 2017

cites work

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Prion diseases of humans and animals: their causes and molecular basis

1 reference

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7 January 2021

Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

New variant prion protein in a Japanese family with Gerstmann-Sträussler syndrome

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Über eine eigenartige hereditär- familiäre Erkrankung des Zentralnervensystems

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

[An unusual hereditary familial disease of the central nervous system]

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Prion protein mutation in family first reported by Gerstmann, Sträussler, and Scheinker

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Gerstmann-Sträussler-Scheinker disease. II. Neurofibrillary tangles and plaques with PrP-amyloid coexist in an affected family

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

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

1 reference

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7 January 2021

Gerstmann-Sträussler-Scheinker disease. I. Extending the clinical spectrum.

1 reference

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7 January 2021

Gerstmann-Sträussler-Scheinker disease and the Indiana kindred

1 reference

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7 January 2021

Familial Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Beta PP participates in PrP-amyloid plaques of Gerstmann-Sträussler-Scheinker disease, Indiana kindred.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

A68 is a component of paired helical filaments of Gerstmann-Sträussler-Scheinker disease, Indiana kindred.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Proteinase-K-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease (Indiana kindred).

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Phenotypic variability of Gerstmann-Sträussler-Scheinker disease is associated with prion protein heterogeneity.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Sträussler-Scheinker disease

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Prion proteins with different conformations accumulate in Gerstmann-Sträussler-Scheinker disease caused by A117V and F198S mutations

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

A transmembrane form of the prion protein in neurodegenerative disease

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Truncated forms of the human prion protein in normal brain and in prion diseases.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Prion protein selectively binds copper(II) ions

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Copper binding to the N-terminal tandem repeat region of mammalian and avian prion protein: structural studies using synthetic peptides

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Metal-dependent alpha-helix formation promoted by the glycine-rich octapeptide region of prion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Conformational transitions in peptides containing two putative alpha-helices of the prion protein.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Physical studies of conformational plasticity in a recombinant prion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Amyloid fibrils in Gerstmann-Sträussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant allele

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

A 7-kDa prion protein (PrP) fragment, an integral component of the PrP region required for infectivity, is the major amyloid protein in Gerstmann-Sträussler-Scheinker disease A117V.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Allelic origin of the abnormal prion protein isoform in familial prion diseases

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Identification of the prion protein allotypes which accumulate in the brain of sporadic and familial Creutzfeldt-Jakob disease patients.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231).

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Synthetic peptides homologous to prion protein residues 106-147 form amyloid-like fibrils in vitro

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Gerstmann-Sträussler-Scheinker disease (PRNP P102L): amyloid deposits are best recognized by antibodies directed to epitopes in PrP region 90-165

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Gerstmann-Sträussler-Scheinker disease with the PRNP P102L mutation and valine at codon 129

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

A new inherited prion disease (PrP-P105L mutation) showing spastic paraparesis

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

An inherited prion disease with a PrP P105L mutation: clinicopathologic and PrP heterogeneity

1 reference

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7 January 2021

Inherited prion disease with an alanine to valine mutation at codon 117 in the prion protein gene

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Gerstmann-Sträussler-Scheinker disease in an Alsatian family: clinical and genetic studies

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

A new PRNP mutation (G131V) associated with Gerstmann-Sträussler-Scheinker disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Inherited prion encephalopathy associated with the novel PRNP H187R mutation: a clinical study

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

A case of sporadic Creutzfeldt-Jakob disease with a Gerstmann-Sträussler-Scheinker phenotype but no alterations in the PRNP gene

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

A case of sporadic Creutzfeldt-Jakob disease with a Gerstmann-Sträussler-Scheinker phenotype but no alterations in the PRNP gene

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Immortality of cell cultures derived from brains of mice and hamsters infected with Creutzfeldt-Jakob disease agent

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Experimental transmission of Creutzfeldt-Jakob disease and related diseases to rodents

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0272-2712%2802%2900064-1

7 January 2021

Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58

40

1 reference

https://api.crossref.org/works/10.1016/S0272-2712(02)00064-1

The phenotypic expression of different mutations in transmissible human spongiform encephalopathy

30 December 2023

62

1 reference

https://api.crossref.org/works/10.1016/S0272-2712(02)00064-1

30 December 2023

Identifiers

1 reference

6 August 2017

1 reference