Case Report: Histopathology and Prion Protein Molecular Properties in Inherited Prion Disease With a

Prion protein (PrP) conformation genetic histopathology insertion mutation

Journal

Frontiers in cellular neuroscience
ISSN: 1662-5102
Titre abrégé: Front Cell Neurosci
Pays: Switzerland
ID NLM: 101477935

Informations de publication

Date de publication:
2020
Historique:
received: 17 03 2020
accepted: 05 05 2020
entrez: 1 8 2020
pubmed: 1 8 2020
medline: 1 8 2020
Statut: epublish

Résumé

The insertion of additional 168 base pair containing seven octapeptide repeats in the prion protein (PrP) gene region spanning residues 51-91 is associated with inherited prion disease. In 2008, we reported the clinical features of a novel

Identifiants

pubmed: 32733203
doi: 10.3389/fncel.2020.00150
pmc: PMC7362343
doi:

Types de publication

Journal Article

Langues

eng

Pagination

150

Subventions

Organisme : NIDDK NIH HHS
ID : K01 DK107787
Pays : United States

Informations de copyright

Copyright © 2020 Cali, Cracco, Saracino, Occhipinti, Coppola, Appleby and Puoti.

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Auteurs

Ignazio Cali (I)

Department of Pathology, School of Medicine, Case Western Reserve University, Cleveland, OH, United States.
National Prion Disease Pathology Surveillance Center (NPDPSC), School of Medicine, Case Western Reserve University, Cleveland, OH, United States.

Laura Cracco (L)

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

Dario Saracino (D)

Division of Neurology, University of Campania "Luigi Vanvitelli", Caserta, Italy.
Prion Disease Diagnosis and Surveillance Center (PDDSC), University of Campania "Luigi Vanvitelli", Caserta, Italy.

Rossana Occhipinti (R)

Department of Physiology and Biophysics, School of Medicine, Case Western Reserve University, Cleveland, OH, United States.

Cinzia Coppola (C)

Division of Neurology, University of Campania "Luigi Vanvitelli", Caserta, Italy.

Brian Stephen Appleby (BS)

Department of Pathology, School of Medicine, Case Western Reserve University, Cleveland, OH, United States.
National Prion Disease Pathology Surveillance Center (NPDPSC), School of Medicine, Case Western Reserve University, Cleveland, OH, United States.
Department of Neurology, School of Medicine, Case Western Reserve University, Cleveland, OH, United States.
Department of Psychiatry, School of Medicine, Case Western Reserve University, Cleveland, OH, United States.

Gianfranco Puoti (G)

Division of Neurology, University of Campania "Luigi Vanvitelli", Caserta, Italy.
Prion Disease Diagnosis and Surveillance Center (PDDSC), University of Campania "Luigi Vanvitelli", Caserta, Italy.

Classifications MeSH