基因名：
FBP1
产品别名：
FBP； FBP1； fructose-bisphosphatase 1； fructose-bisphosphatase 1； fructose-1,6-bisphosphatase 1； D-fructose-1,6-bisphosphate 1-phosphohydrolase 1； FBPase 1； growth-inhibiting protein 17； liver FBPase； 果糖1，6-二磷酸酯酶； 果糖二磷酸酶1；
背景信息：
Fructose-1,6-Bisphosphatase 1 (FBPase 1) is a member of the FBPase class 1 family. FBPase 1 is a gluconeogenesis regulatory protein, which catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate and inorganic phosphate. FBPase 1 can assume an active R-state, or an inactive T-state. FBPase 1 deficiency is inherited as an autosomal recessive disorder mainly in the liver and causes life-threatening episodes of hypoglycemia and metabolic acidosis in newborn infants or young children. FBPase 1 coupled with phosphofructokinase (PFK) is involved in the metabolism of pancreatic islet cells.
