A novel V272D presenilin mutation associated with logopenia, disorientation, and apraxia in an autosomal-dominant Alzheimer's disease family.


Journal

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

Informations de publication

Date de publication:
01 2020
Historique:
received: 07 05 2019
revised: 26 06 2019
accepted: 04 07 2019
pubmed: 11 9 2019
medline: 12 9 2020
entrez: 11 9 2019
Statut: ppublish

Résumé

In the present study, a novel mutation in the presenilin 1 gene was discovered in an Iraq-native patient with early-onset Alzheimer's disease, who presented with speech impairment and memory decline at age 46 years. Magnetic resonance imaging showed a frontotemporal atrophy. Sanger sequencing identified a heterozygous T to A transversion at position 815 (c.815T>A) in the presenilin 1 gene (PSEN1), resulting in a novel missense mutation at codon 272 from valine to aspartate (V272D). We tested this PSEN1 mutation in vitro and found V272D resulted in an altered Aβ42/40 ratio.

Identifiants

pubmed: 31500908
pii: S0197-4580(19)30204-0
doi: 10.1016/j.neurobiolaging.2019.07.002
pii:
doi:

Substances chimiques

Amyloid beta-Peptides 0
PSEN1 protein, human 0
Peptide Fragments 0
Presenilin-1 0
amyloid beta-protein (1-40) 0
amyloid beta-protein (1-42) 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

154.e5-154.e7

Subventions

Organisme : NIA NIH HHS
ID : RF1 AG006173
Pays : United States

Informations de copyright

Copyright © 2019 Elsevier Inc. All rights reserved.

Auteurs

David Mengel (D)

Chair of Geriatric Medicine, University Essen, and Geriatric Centre Haus Berge, Contilia Group, Essen, Germany; Department of Neurology, Philipps-University Marburg, Marburg, Germany; Laboratory for Neurodegenerative Research, Ann Romney Center for Neurologic Diseases, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

Lei Liu (L)

Laboratory for Neurodegenerative Research, Ann Romney Center for Neurologic Diseases, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

Raina Yamamoto (R)

Medizinisches Versorgungszentrum Dr. Eberhard & Partner, Dortmund, Germany.

Stefan Zülow (S)

Institute for Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, Essen, Germany.

Cornelius Deuschl (C)

Institute for Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, Essen, Germany.

Dirk M Hermann (DM)

Department of Neurology, University Hospital Essen, Essen, Germany.

Inga Zerr (I)

Department of Neurology, University of Göttingen, Göttingen, Germany.

Dennis J Selkoe (DJ)

Laboratory for Neurodegenerative Research, Ann Romney Center for Neurologic Diseases, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

Richard Dodel (R)

Chair of Geriatric Medicine, University Essen, and Geriatric Centre Haus Berge, Contilia Group, Essen, Germany; Department of Neurology, Philipps-University Marburg, Marburg, Germany. Electronic address: richard.dodel@uk-essen.de.

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Classifications MeSH