Hepatic fructose-1,6-bisphosphatase (FBP1) is a gluconeogenesis regulatory enzyme which catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate and inorganic phosphate.
Hepatic fructose-1,6-bisphosphatase (FBP1) is a gluconeogenesis regulatory enzyme which catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate and inorganic phosphate.
The FBP1 gene, located at chromosome 9q22.2-q22.3, contains 7 exons and spans more than 31 kb. The mature protein produced by this genes consists of 338 amino acids.
Mutation of the FBP1 gene causes deficiency of fructose-1,6-bisphosphatase which is associated with fasting hypoglycemia and metabolic acidosis due to impaired gluconeogenesis.