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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 Author contributions YM S conceived the study and revised the manuscript

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10.1515/jbcpp-2020-0107 J Basic Clin Physiol Pharmacol

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