Permanent neonatal diabetes-causing insulin mutations have dominant negative effects on beta cell identity

• Main Authors: Yuwei Zhang (Author), Lina Sui (Author), Qian Du (Author), Leena Haataja (Author), Yishu Yin (Author), Ryan Viola (Author), Shuangyi Xu (Author), Christian Ulrik Nielsson (Author), Rudolph L. Leibel (Author), Fabrizio Barbetti (Author), Peter Arvan (Author), Dieter Egli (Author)
• Format: Book
• Published: Elsevier, 2024-02-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Objective: Heterozygous coding sequence mutations of the INS gene are a cause of permanent neonatal diabetes (PNDM), requiring insulin therapy similar to T1D. While the negative effects on insulin processing and secretion are known, how dominant insulin mutations result in a continued decline of bet...