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Functional evaluation of insulin secretion of islets isolated from GCK-HI patients. Perifusion of primary islets isolated from surgical pancreatic specimens from GCK-HI patients (W99C, S69_E70insVPL, or R447L) to assess insulin release compared with normal control subjects. Stimulated with glucose ramp (0–25 mmol/L) and KCl (30 mmol/L) (A), AAM ramp (0–12 mmol/L) and KCl (30 mmol/L) (B), or glyburide (0.3 μmol/L) (C). Glucose threshold is indicated by dashed vertical lines.
