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Abbreviations CHI: Congenital hyperinsulinism GDH-HI: Glutamate dehydrogenase hyperinsulinism GDH: Glutamate dehydrogenase GLUD1: Glutamate dehydrogenase 1 GLUT2: Glucose transporter 2 GCK: Glucokinase G6P: Glucose-6-phosphate TCA: Tricarboxylic acid KATP: ATP-sensitive potassium GTP: Guanosine triphosphate NADH: Nicotinamide adenine diphosphate hydride ADP: Adenosine diphosphate ATP: Adenosine triphosphate HI/HA: Hyperinsulinism/hyperammonemia HHF6: Familial hyperinsulinemic hypoglycemia-6 -KG: Alpha-ketoglutaric acid DES: Diethylstilbestrol EGCG: Epigallocatechin gallate SIRT4: Silent information regulator 4, Sirtuin 4 MCD: Malonyl coenzyme A decarboxylase NAG: N-acetylglutamate CPS: Carbamoyl phosphate synthetase BCH: 2-Aminobicyclo [2, 2, 1] heptane-2-carboxylic acid GABA: -Aminobutyric acid References Yorifuji T