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

doi:10.1074/jbc.M113.470948

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

Department of Physiology

Research output: Contribution to journal › Article › peer-review

42 Citations (Scopus)

Abstract

Background: Defective folding and trafficking of β-cell ATP-sensitive potassium (K_ATP) channels causes congenital hyperinsulinism. Results: Carbamazepine improves the processing and surface expression of trafficking-impaired K_ATP 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 K_ATPchannel trafficking.

Original language	English
Pages (from-to)	20942-20954
Number of pages	13
Journal	Journal of Biological Chemistry
Volume	288
Issue number	29
DOIs	https://doi.org/10.1074/jbc.M113.470948
Publication status	Published - 2013 Jul 19

All Science Journal Classification (ASJC) codes

Biochemistry
Molecular Biology
Cell Biology

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10.1074/jbc.M113.470948

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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.",

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Carbamazepine as a novel small molecule corrector of trafficking-impaired atp-sensitive potassium channels identified in congenital hyperinsulinism. / Chen, Pei Chun; Olson, Erik M.; Zhou, Qing; Kryukova, Yelena; Sampson, Heidi M.; Thomas, David Y.; Shyng, Show Ling.

In: Journal of Biological Chemistry, Vol. 288, No. 29, 19.07.2013, p. 20942-20954.

Research output: Contribution to journal › Article › peer-review

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AU - Kryukova, Yelena

AU - Sampson, Heidi M.

AU - Thomas, David Y.

AU - Shyng, Show Ling

PY - 2013/7/19

Y1 - 2013/7/19

N2 - 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.

AB - 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.

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

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