Generation of three induced pluripotent stem cell lines from individuals with Aicardi-Goutières syndrome caused by a c.3019G>A (p.G1007R) autosomal dominant pathogenic variant in ADAR1.


Journal

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

Informations de publication

Date de publication:
28 Dec 2023
Historique:
received: 20 10 2023
revised: 21 12 2023
accepted: 26 12 2023
medline: 6 1 2024
pubmed: 6 1 2024
entrez: 5 1 2024
Statut: aheadofprint

Résumé

Mutations in Adenosine deaminase acting on RNA 1 (ADAR1) gene encoding RNA editing enzyme ADAR1 results in the neuroinflammatory leukodystrophy Aicardi Goutières Syndrome (AGS). AGS is an early onset leukoencephalopathy with an exacerbated interferon response leading to neurological regression with intellectual disability, spasticity, and motor deficits. We have generated three induced pluripotent stem cell (iPSC) lines from peripheral blood mononuclear cells (PBMCs) of individuals with ADAR1

Identifiants

pubmed: 38181636
pii: S1873-5061(23)00285-4
doi: 10.1016/j.scr.2023.103299
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

103299

Informations de copyright

Copyright © 2023. Published by Elsevier B.V.

Déclaration de conflit d'intérêts

Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Adeline Vanderver reports financial support was provided by Commonwealth Universal Research Enhancement (C.U.R.E.), U01 (NS106845) and R21 Grant. Adeline Vanderver Eli Lilly, Boehringer Ingelhiem, Gilead, Biogen, Illumina, Takeda, Orchard, Passage Bio, Homology, Myrtelle, Synaptixbio, Sanofi, Sana, Affinia related to work in the leukodystrophies.

Auteurs

Luis Garcia (L)

Department of Neurology, The Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Carlos Dominguez Gonzalez (CD)

Department of Neurology, The Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Alyssa Gagne (A)

Penn Institute for Regenerative Medicine, University of Pennsylvania, Philadelphia, PA, United States.

Jean Ann McGuire (JA)

Department of Pathology and Lab Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Deborah French (D)

Department of Pathology and Lab Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Asako Takanohashi (A)

Department of Neurology, The Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Akshata Almad (A)

Department of Neurology, The Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Adeline Vanderver (A)

Department of Neurology, The Children's Hospital of Philadelphia, Philadelphia, PA, United States. Electronic address: vandervera@chop.edu.

Sunetra Sase (S)

Department of Neurology, The Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Classifications MeSH