Generation of induced pluripotent stem cells (iPSCs) from a microvillus inclusion disease patient with a homozygous missense mutation in UNC45A.


Journal

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

Informations de publication

Date de publication:
04 2023
Historique:
received: 03 08 2022
revised: 03 02 2023
accepted: 23 02 2023
pubmed: 4 3 2023
medline: 21 3 2023
entrez: 3 3 2023
Statut: ppublish

Résumé

Mutations in UNC45A, a co-chaperone for myosins, were recently found causative of a syndrome combining cholestasis, diarrhea, loss of hearing and bone fragility. We generated induced pluripotent stem cells (iPSCs) from a patient with a homozygous missense mutation in UNC45A. Cells from this patient, which were reprogrammed using integration-free Sendaï virus, have normal karyotype, express pluripotency markers and are able to differentiate into the three germ cell layers.

Identifiants

pubmed: 36868038
pii: S1873-5061(23)00043-0
doi: 10.1016/j.scr.2023.103057
pii:
doi:

Substances chimiques

UNC45A protein, human 0
Intracellular Signaling Peptides and Proteins 0

Types de publication

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

Langues

eng

Sous-ensembles de citation

IM

Pagination

103057

Informations de copyright

Copyright © 2023 The Author(s). 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

Celine Banal (C)

Université Paris Cité, Imagine Institute, INSERM U1163, iPS Core Facility, 75015 Paris, France.

Eddy Quelennec (E)

Université Paris Cité, Imagine Institute, INSERM U1163, iPS Core Facility, 75015 Paris, France.

Cécile Talbotec (C)

Department of Pediatric Gastroenterology, Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, F-75015 Paris, France.

Anis Khiat (A)

Université Paris Cité, Imagine Institute, INSERM U1163 and Laboratory of Intestinal Immunity, 75015 Paris, France.

Fabienne Charbit-Henrion (F)

Department of Pediatric Gastroenterology, Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, F-75015 Paris, France; Department of Genomic Medecine for Rare Diseases, Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, F-75015 Paris, France.

Nadine Cerf-Bensussan (N)

Université Paris Cité, Imagine Institute, INSERM U1163 and Laboratory of Intestinal Immunity, 75015 Paris, France.

Nathalie Lefort (N)

Université Paris Cité, Imagine Institute, INSERM U1163, iPS Core Facility, 75015 Paris, France. Electronic address: nathalie.lefort@inserm.fr.

Corinne Lebreton (C)

Université Paris Cité, Imagine Institute, INSERM U1163 and Laboratory of Intestinal Immunity, 75015 Paris, France. Electronic address: corinne.lebreton@inserm.fr.

Articles similaires

[Redispensing of expensive oral anticancer medicines: a practical application].

Lisanne N van Merendonk, Kübra Akgöl, Bastiaan Nuijen
1.00
Humans Antineoplastic Agents Administration, Oral Drug Costs Counterfeit Drugs

Smoking Cessation and Incident Cardiovascular Disease.

Jun Hwan Cho, Seung Yong Shin, Hoseob Kim et al.
1.00
Humans Male Smoking Cessation Cardiovascular Diseases Female
Humans United States Aged Cross-Sectional Studies Medicare Part C
1.00
Humans Yoga Low Back Pain Female Male

Classifications MeSH