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

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. © 2013 by The American Society for Biochemistry and Molecular Biology, Inc.