Integrative Physiology

Pflügers Archiv - European Journal of Physiology

, Volume 460, Issue 2, pp 307-320

First online:

KATP channelopathies in the pancreas

  • Maria S. RemediAffiliated withDepartment of Cell Biology and Physiology, Washington University School of Medicine Email author 
  • , Joseph C. KosterAffiliated withDepartment of Cell Biology and Physiology, Washington University School of Medicine

Rent the article at a discount

Rent now

* Final gross prices may vary according to local VAT.

Get Access

Abstract

Adenosine-triphosphate-sensitive potassium channels (KATP) are regulated by adenosine nucleotides, and, thereby, couple cellular metabolism with electrical activity in multiple tissues including the pancreatic β-cell. The critical involvement of KATP in insulin secretion is confirmed by the demonstration that inactivating and activating mutations in KATP underlie persistent hyperinsulinemia and neonatal diabetes mellitus, respectively, in both animal models and humans. In addition, a common variant in KATP represents a risk factor in the etiology of type 2 diabetes. This review focuses on the mechanistic basis by which KATP mutations underlie insulin secretory disorders and the implications of these findings for successful clinical intervention.

Keywords

Neonatal Diabetes Hyperinsulinism Diazoxide Sulfonylurea Kir6.2 SUR1