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Rethinking HNF1A-MODY: HNF1A at the crossroads of development and multiorgan metabolic disease.
Genes Dev., DOI: 10.1101/gad.353709.126 (2026)
In this issue of Genes & Development, Unger and colleagues (doi:10.1101/gad.353153.125) combined human pluripotent stem cell-derived in vitro models with targeted in vivo mouse models to reveal multiple developmental defects triggered by HNF1A mutations causing maturity-onset diabetes of the young. This work paints the picture of a disorder that starts well before diabetes manifests, highlighting its complexity arising from the diverse roles of HNF1A across distinct cell types, each potentially differentially impacted by different mutations.
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Publication type
Article: Journal article
Document type
Comment, Opinion
Keywords
Gli3 ; Hnf1a ; Duodenal Fate ; Intestinal Elongation ; Pancreatic Fate ; Pancreatic Progenitors
ISSN (print) / ISBN
0890-9369
e-ISSN
1549-5477
Journal
Genes and Development
Publisher
Cold Spring Harbor Laboratory Press
Reviewing status
Peer reviewed
Institute(s)
Institute of Pancreatic Islet Research (IPI)