Article
Effects of a GTP-insensitive mutation of glutamate dehydrogenase on insulin secretion in transgenic mice.
The Journal of biological chemistry - 2 Jun 2006
Li Changhong, Matter Andrea, Kelly Andrea, Petty Thomas J, Najafi Habiba, MacMullen Courtney, Daikhin Yevgeny, Nissim Ilana, Lazarow Adam, Kwagh Jae, Collins Heather W, Hsu Betty Y L, Nissim Itzhak, Yudkoff Marc, Matschinsky Franz M, Stanley Charles A
Abstract excerpt
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 beta-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...
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