Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments.

Aggregate formation Creutzfeldt-Jakob disease Epitope mapping Mass spectrometry Multimers Prion protein

Journal

Acta neuropathologica communications
ISSN: 2051-5960
Titre abrégé: Acta Neuropathol Commun
Pays: England
ID NLM: 101610673

Informations de publication

Date de publication:
29 05 2019
Historique:
received: 22 04 2019
accepted: 09 05 2019
entrez: 31 5 2019
pubmed: 31 5 2019
medline: 22 4 2020
Statut: epublish

Résumé

Despite their phenotypic heterogeneity, most human prion diseases belong to two broadly defined groups: Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker disease (GSS). While the structural characteristics of the disease-related proteinase K-resistant prion protein (resPrP

Identifiants

pubmed: 31142381
doi: 10.1186/s40478-019-0734-2
pii: 10.1186/s40478-019-0734-2
pmc: PMC6540574
doi:

Substances chimiques

PrPSc Proteins 0

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

85

Subventions

Organisme : NIA NIH HHS
ID : P30 AG010133
Pays : United States
Organisme : NIAID NIH HHS
ID : P01 AI077774
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS083687
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS103848
Pays : United States
Organisme : NIAID NIH HHS
ID : P01 AI106705
Pays : United States

Commentaires et corrections

Type : ErratumIn

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Auteurs

Laura Cracco (L)

Department of Pathology, Case Western Reserve University, Cleveland, OH, USA.

Xiangzhu Xiao (X)

Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH, USA.

Satish K Nemani (SK)

Department of Pathology, Case Western Reserve University, Cleveland, OH, USA.

Jody Lavrich (J)

Department of Pathology, Case Western Reserve University, Cleveland, OH, USA.

Ignazio Cali (I)

Department of Pathology, Case Western Reserve University, Cleveland, OH, USA.
National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH, USA.

Bernardino Ghetti (B)

Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.

Silvio Notari (S)

Department of Pathology, Case Western Reserve University, Cleveland, OH, USA.

Witold K Surewicz (WK)

Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH, USA.

Pierluigi Gambetti (P)

Department of Pathology, Case Western Reserve University, Cleveland, OH, USA. pxg13@case.edu.

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Classifications MeSH