Functional and Metabolomic Consequences of K_ATP Channel Inactivation in Human Islets.

ABSTRACT: Loss-of-function mutations of β-cell K_ATP channels cause the most severe form of congenital hyperinsulinism (K_ATPHI). K_ATPHI is characterized by fasting and protein-induced hypoglycemia that is unresponsive to medical therapy. For a better understanding of the pathophysiology of K_ATPHI, we examined cytosolic calcium ([Ca²⁺] _i ), insulin secretion, oxygen consumption, and [U-¹³C]glucose metabolism in islets isolated from the pancreases of children with K_ATPHI who required pancreatectomy. Basal [Ca²⁺] _i and insulin secretion were higher in K_ATPHI islets compared with controls. Unlike controls, insulin secretion in K_ATPHI islets increased in respons

SUBMITTER: Li C 

PROVIDER: S-EPMC5482088 | biostudies-literature | 2017 Jul

REPOSITORIES: biostudies-literature