Evidence map›Paper›PMID 42714587›Full record

ArticlePediatric surgery international2026

Characterization of stem cells from exfoliated deciduous teeth from a patient with Alagille syndrome carrying a JAG1 mutation.

Yasuyuki Uchida, Soichiro Sonoda, Lisha Dai, Koichiro Yoshimaru, Satoshi Fukumoto, Haruyoshi Yamaza, Yoshinao Oda, Kenjiro Kosaki, Tatsuro Tajiri, Takayoshi Yamaza and 1 more

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Article in Pediatric surgery international, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

11 authors.

Yasuyuki UchidaDepartment of Pediatric Surgery, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Soichiro SonodaDepartment of Molecular Cell Biology and Oral Anatomy, Kyushu University Graduate School of Dental Science, Fukuoka, 812-8582, Japan.
Lisha DaiDepartment of Molecular Cell Biology and Oral Anatomy, Kyushu University Graduate School of Dental Science, Fukuoka, 812-8582, Japan.
Koichiro YoshimaruDepartment of Pediatric Surgery, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Satoshi FukumotoSection of Pediatric Dentistry, Faculty of Dental Science, Kyushu University, Fukuoka, 812-8582, Japan.
Haruyoshi YamazaDepartment of Pediatric Dentistry, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, 890-8544, Japan.
Yoshinao OdaDepartment of Anatomic Pathology, Kyushu University Graduate School of Medical Sciences, Fukuoka, 812-8582, Japan.
Kenjiro KosakiCenter for Medical Genetics, Keio University School of Medicine, Tokyo, Japan.
Tatsuro TajiriDepartment of Pediatric Surgery, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Takayoshi YamazaDepartment of Molecular Cell Biology and Oral Anatomy, Kyushu University Graduate School of Dental Science, Fukuoka, 812-8582, Japan.
Toshiharu MatsuuraDepartment of Pediatric Surgery, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. matsuura.toshiharu.809@m.kyushu-u.ac.jp.

Funding

Japan Society for the Promotion of Science JP21K16932Japan Society for the Promotion of Science JP22K19565
6 · The paper itself

Abstract

purposeAlagille syndrome (ALGS) is an autosomal dominantly inherited disorder primarily caused by mutations in the Jagged Canonical Notch Ligand 1 (JAG1) gene. Although many pluripotent stem cells are well established, no patient-derived stem cells from exfoliated deciduous teeth (SHED) have been developed. In this study, we aimed to establish SHED from an ALGS patient carrying a heterozygous JAG1

methodsWe isolated SHED from a deciduous tooth of an ALGS patient with a heterozygous JAG1 mutation (ALGS-SHED) by the colony-forming unit-fibroblast (CFU-F) method. We then compared the characteristics of ALGS-SHED and healthy donor-derived control SHED (CONT-SHED).

resultsALGS-SHED displayed mesenchymal stem cell features as indicated by CFU-F formation, immunophenotype, and mesenchymal multipotency into adipocytes, chondrocytes, and osteoblasts. ALGS-SHED showed reduced population doubling capacity and exhibited induced chondrogenic potency and diminished osteogenic potency, but similar hepatic potency. ALGS-SHED damaged in situ potency to form bile duct-like tubular structures in the livers of chronically CCl

conclusionsWe successfully established ALGS-SHED from an ALGS patient carrying a heterozygous JAG1 mutation. Our established ALGS-SHED may represent a potential model for studying ALGS involving a JAG1 mutation.

Indexed as

Alagille SyndromeIntercellular Signaling Peptides and ProteinsJagged-1 ProteinMembrane ProteinsMesenchymal Stem CellsMutationStem CellsTooth, DeciduousAnimalsCells, CulturedHumansMaleMiceSerrate-Jagged ProteinsTooth ExfoliationIntercellular Signaling Peptides and ProteinsJAG1 protein, humanJagged-1 ProteinMembrane ProteinsSerrate-Jagged ProteinsAlagille syndromeHepatobiliary potencyJAG1 mutationStem cells exfoliated deciduous teeth

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.