UniProt Protein Function: | 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. |
UniProt Protein Details: | Protein type:Phosphatase (non-protein); Carbohydrate Metabolism - pentose phosphate pathway; EC 3.1.3.11; Carbohydrate Metabolism - fructose and mannose; Mitochondrial; Carbohydrate Metabolism - glycolysis and gluconeogenesis 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; negative regulation of Ras protein signal transduction; glucose metabolic process; regulation of gluconeogenesis; pathogenesis; negative regulation of cell growth; negative regulation of glycolysis; fructose 6-phosphate metabolic process; protein homotetramerization; fructose metabolic process; gluconeogenesis Disease: Fructose-1,6-bisphosphatase Deficiency |
NCBI Summary: | 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] |
UniProt Code: | P09467 |
NCBI GenInfo Identifier: | 311033495 |
NCBI Gene ID: | 2203 |
NCBI Accession: | P09467.5 |
UniProt Secondary Accession: | P09467,O75571, Q53F94, Q96E46, |
UniProt Related Accession: | P09467 |
Molecular Weight: | 338 |
NCBI Full Name: | Fructose-1,6-bisphosphatase 1 |
NCBI Synonym Full Names: | fructose-1,6-bisphosphatase 1 |
NCBI Official Symbol: | FBP1 |
NCBI Official Synonym Symbols: | FBP |
NCBI Protein Information: | fructose-1,6-bisphosphatase 1; FBPase 1; liver FBPase; fructose-bisphosphatase 1; growth-inhibiting protein 17; D-fructose-1,6-bisphosphate 1-phosphohydrolase 1 |
UniProt Protein Name: | Fructose-1,6-bisphosphatase 1 |
UniProt Synonym Protein Names: | D-fructose-1,6-bisphosphate 1-phosphohydrolase 1; Liver FBPase |
Protein Family: | Fat-body protein |
UniProt Gene Name: | FBP1 |
UniProt Entry Name: | F16P1_HUMAN |