FBP1
Function
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.
Involvement in disease
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.
None
The disease is caused by variants affecting the gene represented in this entry.
Pathway
Carbohydrate biosynthesis; gluconeogenesis.
Sequence Similarities
Belongs to the FBPase class 1 family.
Tissue Specificity
Expressed in pancreatic islets.
Alternative names
FBP, FBP1, FBPase 1, Liver FBPase