Generation of three induced pluripotent stem cell lines (UQACi003-A, UQACi004-A, and UQACi006-A) from three patients with KRT5 epidermolysis bullosa simplex mutations.


Journal

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

Informations de publication

Date de publication:
04 2022
Historique:
received: 05 12 2021
revised: 28 01 2022
accepted: 23 02 2022
pubmed: 6 3 2022
medline: 16 4 2022
entrez: 5 3 2022
Statut: ppublish

Résumé

Heterozygous mutations within Keratin 5 (KRT5) are common genetic causes of epidermolysis bullosa simplex (EBS), a skin fragility disorder characterized by blisters, which appear after minor trauma. Using CytoTune®Sendai virus, we generated three human induced pluripotent stem cell (iPSC) lines from three EBS patients carrying respectively the single heterozygous mutations in KRT5, c.449 T > C, c.980 T > C, and c.608 T > C. All lines display normal karyotype, expressed high levels of pluripotent markers, and can differentiate into derivatives of the three germ layers. These iPSCs are helpful for a better understanding of the EBS pathogenesis and developing novel therapeutic approaches.

Identifiants

pubmed: 35247839
pii: S1873-5061(22)00075-7
doi: 10.1016/j.scr.2022.102726
pii:
doi:

Substances chimiques

KRT5 protein, human 0
Keratin-5 0

Types de publication

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

Langues

eng

Sous-ensembles de citation

IM

Pagination

102726

Informations de copyright

Copyright © 2022 The Author(s). Published by Elsevier B.V. All rights reserved.

Auteurs

Mbarka Bchetnia (M)

Université du Québec à Chicoutimi, Département des sciences fondamentales, Saguenay, QC, Canada.

Laurie Martineau (L)

Centrer hospitalier universitaire (CHU) de Québec, Université Laval, Québec, QC, Canada.

Véronique Racine (V)

Centrer hospitalier universitaire (CHU) de Québec, Université Laval, Québec, QC, Canada.

Julie Powell (J)

CHU Sainte-Justine, Université de Montréal, Montréal, QC, Canada.

Catherine McCuaig (C)

CHU Sainte-Justine, Université de Montréal, Montréal, QC, Canada.

Charles Morin (C)

Université du Québec à Chicoutimi, Département des sciences fondamentales, Saguenay, QC, Canada; Centre intégré universitaire de santé et de services sociaux (CIUSSS) du Saguenay-Lac-Saint-Jean, Saguenay, QC, Canada.

Audrey Dupérée (A)

Centre intégré universitaire de santé et de services sociaux (CIUSSS) du Saguenay-Lac-Saint-Jean, Saguenay, QC, Canada.

François Gros-Louis (F)

Centrer hospitalier universitaire (CHU) de Québec, Université Laval, Québec, QC, Canada.

Catherine Laprise (C)

Université du Québec à Chicoutimi, Département des sciences fondamentales, Saguenay, QC, Canada; Centre intégré universitaire de santé et de services sociaux (CIUSSS) du Saguenay-Lac-Saint-Jean, Saguenay, QC, Canada. Electronic address: catherine.laprise@uqac.ca.

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Classifications MeSH