FBP1 Antibody – Rabbit Polyclonal

FBPase: a key enzyme of gluconeogenesis that catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate, a precursor to glucose 6-phosphate. A regulator of glucose synthesis from non-carbohydrates. Two paralogs of this enzyme exists in humans, FBP1 in the liver and FBP2 in muscle. While both forms are inhibited allosterically by AMP, NAD and Ca2+, the muscle form is about 100-fold more sensitive to AMP and NAD, and about 1000-fold more sensitive to inhibition by Ca2+. Forms homotetramers that are stabilized in the active state by divalent cations (Mg2+, Mn2+ , Co+2, or Zn2+). Deficiency of FBP1 leads to a disorder mainly in the liver and causes life-threatening episodes of hypoglycemia and metabolic acidosis (lactacidemia) in newborn infants or young children.

Protein type: Carbohydrate Metabolism - glycolysis and gluconeogenesis; Phosphatase (non-protein); EC 3.1.3.11; Mitochondrial; Carbohydrate Metabolism - pentose phosphate pathway; Carbohydrate Metabolism - fructose and mannose

Chromosomal Location of Human Ortholog: 9q22.3

Cellular Component: cytoplasm; cytosol

Molecular Function: monosaccharide binding; identical protein binding; protein binding; metal ion binding; fructose-bisphosphatase activity; AMP binding

Biological Process: dephosphorylation; carbohydrate metabolic process; glucose metabolic process; negative regulation of Ras protein signal transduction; regulation of gluconeogenesis; pathogenesis; negative regulation of cell growth; fructose 6-phosphate metabolic process; negative regulation of glycolysis; fructose metabolic process; protein homotetramerization; gluconeogenesis

Disease: Fructose-1,6-bisphosphatase Deficiency