Carbamazepine as a novel small molecule corrector of trafficking-impaired atp-sensitive potassium channels identified in congenital hyperinsulinism

Pei Chun Chen, Erik M. Olson, Qing Zhou, Yelena Kryukova, Heidi M. Sampson, David Y. Thomas, Show Ling Shyng

Research output: Contribution to journalArticlepeer-review

40 Citations (Scopus)

Abstract

Background: Defective folding and trafficking of β-cell ATP-sensitive potassium (KATP) channels causes congenital hyperinsulinism. Results: Carbamazepine improves the processing and surface expression of trafficking-impaired KATP channels harboring a subset of sulfonylurea receptor 1 mutations. Conclusion: Carbamazepine is a novel corrector of KATP channels. Significance: Carbamazepine may be used to treat congenital hyperinsulinism caused by defective KATPchannel trafficking.

Original languageEnglish
Pages (from-to)20942-20954
Number of pages13
JournalJournal of Biological Chemistry
Volume288
Issue number29
DOIs
Publication statusPublished - 2013 Jul 19

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Molecular Biology
  • Cell Biology

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