[HTML][HTML] Effects of a GTP-insensitive Mutation of Glutamate Dehydrogenase on Insulin Secretion in Transgenic Mice*♦
Glutamate dehydrogenase (GDH) plays an important role in insulin secretion as evidenced
in children by gain of function mutations of this enzyme that cause a hyperinsulinism-
hyperammonemia syndrome (GDH-HI) and sensitize β-cells to leucine stimulation. GDH
transgenic mice were generated to express the human GDH-HI H454Y mutation and human
wild-type GDH in islets driven by the rat insulin promoter. H454Y transgene expression was
confirmed by increased GDH enzyme activity in islets and decreased sensitivity to GTP …
in children by gain of function mutations of this enzyme that cause a hyperinsulinism-
hyperammonemia syndrome (GDH-HI) and sensitize β-cells to leucine stimulation. GDH
transgenic mice were generated to express the human GDH-HI H454Y mutation and human
wild-type GDH in islets driven by the rat insulin promoter. H454Y transgene expression was
confirmed by increased GDH enzyme activity in islets and decreased sensitivity to GTP …