FBP1 (Fructose-1,6-bisphosphatase 1) is a gluconeogenesis regulatory enzyme that catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate and inorganic phosphate (1). It is a key, rate-controlling enzyme of gluconeogenesis, an important metabolic pathway that allows the cells to synthesize glucose and grow on non-carbohydrate carbon sources, such as glycerol, organic acids and amino acids (2). Fructose-1,6-diphosphatase deficiency is associated with hypoglycemia and metabolic acidosis (1).
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