In humans, a syndrome called hyperinsulinism/hyperammonemia (HI/HA) could be due in part to mutations in the GLUD1 gene [40] that increase the synthesis of α-ketoglutarate leading to an increase of insulin exocytosis in the pancreatic β-cells and consequently to an increase of glucose absorption by the liver. The gene discussed is GLUD1; the disease is Hyperammonemia.