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MGP001041

UniProt Annotations

Entry Information
Gene Namefructose-1,6-bisphosphatase 1
Protein EntryF16P1_HUMAN
UniProt IDP09467
SpeciesHuman
Comments
Comment typeDescription
Biophysicochemical PropertiesKinetic parameters: KM=2.7 uM for fructose 1,6-biphosphate {ECO:0000269|PubMed:17350621}; Note=The kinetic constants are determined for the recombinant enzyme expressed in E.coli.;
Catalytic ActivityD-fructose 1,6-bisphosphate + H(2)O = D- fructose 6-phosphate + phosphate.
CofactorName=Mg(2+); Xref=ChEBI:CHEBI:18420; Evidence={ECO:0000250}; Note=Binds 3 Mg(2+) ions per subunit. {ECO:0000250};
DiseaseFructose-1,6-bisphosphatase deficiency (FBPD) [MIM:229700]: Inherited as an autosomal recessive disorder mainly in the liver and causes life-threatening episodes of hypoglycemia and metabolic acidosis (lactacidemia) in newborn infants or young children. {ECO:0000269|PubMed:12126934, ECO:0000269|PubMed:9382095}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Enzyme RegulationSubject to complex allosteric regulation. The enzyme can assume an active R-state, or an inactive T-state. Intermediate conformations may exist. AMP acts as allosteric inhibitor. AMP binding affects the turnover of bound substrate and not the affinity for substrate. Fructose 2,6-bisphosphate acts as competitive inhibitor. Fructose 2,6-bisphosphate and AMP have synergistic effects. {ECO:0000269|PubMed:16442285, ECO:0000269|PubMed:16446092, ECO:0000269|PubMed:17350621, ECO:0000269|PubMed:18650089, ECO:0000269|PubMed:7809062}.
FunctionCatalyzes 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. {ECO:0000269|PubMed:16497803, ECO:0000269|PubMed:18375435, ECO:0000269|PubMed:22517657}.
InductionUp-regulated in pancreatic islets of individuals with type 2 diabetes. {ECO:0000269|PubMed:18375435}.
InteractionSelf; NbExp=3; IntAct=EBI-712740, EBI-712740; O00499:BIN1; NbExp=4; IntAct=EBI-712740, EBI-719094;
PathwayCarbohydrate biosynthesis; gluconeogenesis.
Sequence CautionSequence=AAC50207.1; Type=Erroneous gene model prediction; Evidence={ECO:0000305};
SimilarityBelongs to the FBPase class 1 family. {ECO:0000305}.
SubunitHomotetramer. {ECO:0000269|PubMed:16442285, ECO:0000269|PubMed:16446092, ECO:0000269|PubMed:18650089, ECO:0000269|PubMed:7809062}.
Tissue SpecificityExpressed in pancreatic islets. {ECO:0000269|PubMed:18375435}.
Web ResourceName=NIEHS-SNPs; URL="http://egp.gs.washington.edu/data/fbp1/";
Web ResourceName=Wikipedia; Note=Fructose bisphosphatase entry; URL="http://en.wikipedia.org/wiki/Fructose_bisphosphatase";
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