A patient with early-onset Alzheimer's disease with a novel PSEN1 p.Leu424Pro mutation.

Early-onset Alzheimer's disease L424P Mutation Novel PSEN1

Journal

Neurobiology of aging
ISSN: 1558-1497
Titre abrégé: Neurobiol Aging
Pays: United States
ID NLM: 8100437

Informations de publication

Date de publication:
12 2019
Historique:
received: 01 03 2019
revised: 14 05 2019
accepted: 18 05 2019
pubmed: 13 7 2019
medline: 5 8 2020
entrez: 13 7 2019
Statut: ppublish

Résumé

"Presenilin 1" (PSEN1) gene mutations are the major known genetic cause of early-onset Alzheimer's disease. Herein, we report a novel heterozygous PSEN1 mutation (p.Leu424Pro) in a Turkish patient presenting with deterioration of short-term memory and visuospatial skills starting at the age of 47 years. This novel mutation is located in the conserved residue of transmembrane domain 8 coded by exon 12. At the protein level, this mutation caused a disruption in the alpha helix structure of PSEN1. The structural and possible functional consequences of the mutation suggest that it has probably a pathogenic effect, which in turns had a potential role in the development of Alzheimer's disease in our patient.

Identifiants

pubmed: 31296348
pii: S0197-4580(19)30170-8
doi: 10.1016/j.neurobiolaging.2019.05.014
pii:
doi:

Substances chimiques

PSEN1 protein, human 0
Presenilin-1 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

238.e1-238.e4

Informations de copyright

Copyright © 2019 Elsevier Inc. All rights reserved.

Auteurs

Gamze Guven (G)

Department of Genetics, Aziz Sancar Institute of Experimental Medicine, Istanbul University, Istanbul, Turkey. Electronic address: zgamzee@gmail.com.

Nihan Erginel-Unaltuna (N)

Department of Genetics, Aziz Sancar Institute of Experimental Medicine, Istanbul University, Istanbul, Turkey.

Bedia Samanci (B)

Department of Neurology, Behavioral Neurology and Movement Disorders Unit, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Cagri Gulec (C)

Department of Medical Genetics, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Hasmet Hanagasi (H)

Department of Neurology, Behavioral Neurology and Movement Disorders Unit, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Basar Bilgic (B)

Department of Neurology, Behavioral Neurology and Movement Disorders Unit, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

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Classifications MeSH