FBP1

Fructose-1,6-bisphosphatase 1, a gluconeogenesis regulatory enzyme, catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate and inorganic phosphate. Fructose-1,6-diphosphatase deficiency is associated with hypoglycemia and metabolic acidosis. [provided by RefSeq, Jul 2008]

Fructose-Bisphosphatase 1

Catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate in the presence of divalent cations, acting as a rate-limiting enzyme in gluconeogenesis. Plays a role in regulating glucose sensing and insulin secretion of pancreatic beta-cells. Appears to modulate glycerol gluconeogenesis in liver. Important regulator of appetite and adiposity; increased expression of the protein in liver after nutrient excess increases circulating satiety hormones and reduces appetite-stimulating neuropeptides and thus seems to provide a feedback mechanism to limit weight gain.

Cellular response to drug Source: UniProtKB
Cellular response to magnesium ion Source: UniProtKB
Dephosphorylation Source: UniProtKB
Fructose 1,6-bisphosphate metabolic process Source: GO_Central
Fructose 6-phosphate metabolic process Source: UniProtKB
Fructose metabolic process Source: GO_Central
Gluconeogenesis Source: UniProtKB
Negative regulation of cell growth Source: UniProtKB
Negative regulation of glycolytic process Source: UniProtKB
Negative regulation of Ras protein signal transduction Source: UniProtKB
Negative regulation of transcription by RNA polymerase II Source: CAFA
Regulation of gluconeogenesis Source: UniProtKB
Sucrose biosynthetic process Source: GO_Central

Cytosol; Extracellular exosome; Nucleus; Cytoplasm

Fructose-1,6-bisphosphatase deficiency (FBP1D):
An autosomal recessive metabolic disorder characterized by impaired gluconeogenesis, and episodes of hypoglycemia and metabolic acidosis that can be lethal in newborn infants or young children.