Correction of the Allan-Herndon-Dudley syndrome-causing SLC16A2 mutation G401R in a patient derived hiPSC line

The X-linked Allan-Herndon-Dudley syndrome (AHDS) is a genetic disorder characterized by severe psychomotor impairment, resulting from mutations in the SLC16A2 gene, which encodes the thyroid hormone transporter MCT8 (monocarboxylate transporter 8). Previously, we established a hiPSC line from a pat...

Full description

Saved in:
Bibliographic Details
Main Authors: Katarzyna A. Ludwik, Robert Opitz, Sabine Jyrch, Matthias Megges, Peter Kühnen, Harald Stachelscheid
Format: Article
Language:English
Published: Elsevier 2025-06-01
Series:Stem Cell Research
Online Access:http://www.sciencedirect.com/science/article/pii/S1873506125000480
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850156421253955584
author Katarzyna A. Ludwik
Robert Opitz
Sabine Jyrch
Matthias Megges
Peter Kühnen
Harald Stachelscheid
author_facet Katarzyna A. Ludwik
Robert Opitz
Sabine Jyrch
Matthias Megges
Peter Kühnen
Harald Stachelscheid
author_sort Katarzyna A. Ludwik
collection DOAJ
description The X-linked Allan-Herndon-Dudley syndrome (AHDS) is a genetic disorder characterized by severe psychomotor impairment, resulting from mutations in the SLC16A2 gene, which encodes the thyroid hormone transporter MCT8 (monocarboxylate transporter 8). Previously, we established a hiPSC line from a patient carrying the SLC16A2:R401G mutation (BIHi045-A). Using CRISPR/Cas9-mediated gene editing, we targeted exon 3 of SLC16A2 and used single-stranded oligodeoxynucleotides as homology-directed repair templates to correct the R401G missense mutation, generating an isogenic control cell line.
format Article
id doaj-art-518bab7ff8f44caba4f49e42422276e9
institution OA Journals
issn 1873-5061
language English
publishDate 2025-06-01
publisher Elsevier
record_format Article
series Stem Cell Research
spelling doaj-art-518bab7ff8f44caba4f49e42422276e92025-08-20T02:24:33ZengElsevierStem Cell Research1873-50612025-06-018510369810.1016/j.scr.2025.103698Correction of the Allan-Herndon-Dudley syndrome-causing SLC16A2 mutation G401R in a patient derived hiPSC lineKatarzyna A. Ludwik0Robert Opitz1Sabine Jyrch2Matthias Megges3Peter Kühnen4Harald Stachelscheid5Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Core Unit Pluripotent Stem Cells and Organoids, 13353 Berlin, GermanyDepartment of Pediatric Endocrinology and Diabetology, Charité - Universitätsmedizin Berlin 13353 Berlin, GermanyDepartment of Pediatric Endocrinology and Diabetology, Charité - Universitätsmedizin Berlin 13353 Berlin, GermanyDepartment of Pediatric Endocrinology and Diabetology, Charité - Universitätsmedizin Berlin 13353 Berlin, GermanyDepartment of Pediatric Endocrinology and Diabetology, Charité - Universitätsmedizin Berlin 13353 Berlin, Germany; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Germany; Corresponding authors.Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Core Unit Pluripotent Stem Cells and Organoids, 13353 Berlin, Germany; Corresponding authors.The X-linked Allan-Herndon-Dudley syndrome (AHDS) is a genetic disorder characterized by severe psychomotor impairment, resulting from mutations in the SLC16A2 gene, which encodes the thyroid hormone transporter MCT8 (monocarboxylate transporter 8). Previously, we established a hiPSC line from a patient carrying the SLC16A2:R401G mutation (BIHi045-A). Using CRISPR/Cas9-mediated gene editing, we targeted exon 3 of SLC16A2 and used single-stranded oligodeoxynucleotides as homology-directed repair templates to correct the R401G missense mutation, generating an isogenic control cell line.http://www.sciencedirect.com/science/article/pii/S1873506125000480
spellingShingle Katarzyna A. Ludwik
Robert Opitz
Sabine Jyrch
Matthias Megges
Peter Kühnen
Harald Stachelscheid
Correction of the Allan-Herndon-Dudley syndrome-causing SLC16A2 mutation G401R in a patient derived hiPSC line
Stem Cell Research
title Correction of the Allan-Herndon-Dudley syndrome-causing SLC16A2 mutation G401R in a patient derived hiPSC line
title_full Correction of the Allan-Herndon-Dudley syndrome-causing SLC16A2 mutation G401R in a patient derived hiPSC line
title_fullStr Correction of the Allan-Herndon-Dudley syndrome-causing SLC16A2 mutation G401R in a patient derived hiPSC line
title_full_unstemmed Correction of the Allan-Herndon-Dudley syndrome-causing SLC16A2 mutation G401R in a patient derived hiPSC line
title_short Correction of the Allan-Herndon-Dudley syndrome-causing SLC16A2 mutation G401R in a patient derived hiPSC line
title_sort correction of the allan herndon dudley syndrome causing slc16a2 mutation g401r in a patient derived hipsc line
url http://www.sciencedirect.com/science/article/pii/S1873506125000480
work_keys_str_mv AT katarzynaaludwik correctionoftheallanherndondudleysyndromecausingslc16a2mutationg401rinapatientderivedhipscline
AT robertopitz correctionoftheallanherndondudleysyndromecausingslc16a2mutationg401rinapatientderivedhipscline
AT sabinejyrch correctionoftheallanherndondudleysyndromecausingslc16a2mutationg401rinapatientderivedhipscline
AT matthiasmegges correctionoftheallanherndondudleysyndromecausingslc16a2mutationg401rinapatientderivedhipscline
AT peterkuhnen correctionoftheallanherndondudleysyndromecausingslc16a2mutationg401rinapatientderivedhipscline
AT haraldstachelscheid correctionoftheallanherndondudleysyndromecausingslc16a2mutationg401rinapatientderivedhipscline