Article
Dysregulation of insulin secretion in children with congenital hyperinsulinism due to sulfonylurea receptor mutations.
Diabetes - 1 Feb 2001
Grimberg A, Ferry R J, Kelly A, Koo-McCoy S, Polonsky K, Glaser B, Permutt M A, Aguilar-Bryan L, Stafford D, Thornton P S, Baker L, Stanley C A
Abstract excerpt
Mutations in the high-affinity sulfonylurea receptor (SUR)-1 cause one of the severe recessively inherited diffuse forms of congenital hyperinsulinism or, when associated with loss of heterozygosity, focal adenomatosis. We hypothesized that SUR1 mutations would render the beta-cell insensitive to sulfonylureas and to glucose. Stimulated insulin responses were compared among eight patients with diffuse...
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