Generation of five induced pluripotent stem cells lines from four members of the same family carrying a C9orf72 repeat expansion and one wild-type member.


Journal

Stem cell research
ISSN: 1876-7753
Titre abrégé: Stem Cell Res
Pays: England
ID NLM: 101316957

Informations de publication

Date de publication:
02 2023
Historique:
received: 26 09 2022
revised: 21 11 2022
accepted: 04 12 2022
pubmed: 18 12 2022
medline: 17 1 2023
entrez: 17 12 2022
Statut: ppublish

Résumé

The most common genetic cause of Amyotrophic Lateral Sclerosis (ALS) is the expansion of a G4C2 hexanucleotide repeat in the C9orf72 gene. The size of the repeat expansion is highly variable and a cut-off of 30 repeats has been suggested as the lower pathological limit. Repeat size variability has been observed intergenerationally and intraindividually in tissues from different organs and within the same tissue, suggesting instability of the pathological repeat expansion. In order to study this genomic instability, we established iPSCs from five members of the same family of which four carried a C9orf72 repeat expansion and one was wild-type.

Identifiants

pubmed: 36528014
pii: S1873-5061(22)00347-6
doi: 10.1016/j.scr.2022.102998
pii:
doi:

Substances chimiques

Proteins 0
C9orf72 Protein 0
C9orf72 protein, human 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

102998

Commentaires et corrections

Type : ErratumIn

Informations de copyright

Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Auteurs

Chiara Lattuada (C)

Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy.

Serena Santangelo (S)

Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy; Department of Medical Biotechnology and Translational Medicine, Università degli Studi di Milano, Milan, Italy.

Silvia Peverelli (S)

Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy.

Philip McGoldrick (P)

Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Canada.

Ekaterina Rogaeva (E)

Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Canada.

Lorne Zinman (L)

Sunnybrook Health Sciences Centre, Toronto, Canada.

Georg Haase (G)

MPATHY Laboratory, Institute of Systems Neuroscience, U1106 INSERM & Aix-Marseille University, Marseille, France.

Vincent Géli (V)

Marseille Cancer Research Centre (CRCM), Inserm U1068, CNRS UMR7258, Institut Paoli-Calmettes, Aix-Marseille University, Marseille, France.

Vincenzo Silani (V)

Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy; "Dino Ferrari" Center, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.

Janice Robertson (J)

Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Canada.

Antonia Ratti (A)

Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy; Department of Medical Biotechnology and Translational Medicine, Università degli Studi di Milano, Milan, Italy.

Patrizia Bossolasco (P)

Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy. Electronic address: p.bossolasco@auxologico.it.

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Classifications MeSH