catalytic activity:L-glutamate + H(2)O + NAD(P)(+) = 2-oxoglutarate + NH(3) + NAD(P)H.,disease:Defects in GLUD1 are the cause of hyperinsulinism-hyperammonemia syndrome (HHS) [MIM:606762]. Elevated oxidation rate of glutamate to alpha-ketoglutarate stimulates insulin secretion in the pancreatic beta cells, while they impair detoxification of ammonium in the liver.,enzyme regulation:Subject to allosteric regulation. Activated by ADP. Inhibited by GTP and ATP. ADP can occupy the NADH binding site and activate the enzyme.,function:May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate.,online information:Glutamate dehydrogenase 1 entry,similarity:Belongs to the Glu/Leu/Phe/Val dehydrogenases family.,subunit:Homohexamer.,
Mitochondrion . Endoplasmic reticulum . Mostly translocates into the mitochondria, only a small amount of the protein localizes to the endoplasmic reticulum. .
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