Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

3.1.3.11

Official Name

fructose-bisphosphatase

Name from literature

hexose diphosphatase

Pathway from literature

reductive pentose phosphate cycle

Pathway from KEGG

Cellular Processes; Endocrine System; Insulin signaling pathway; map04910

Carbohydrate Metabolism; Fructose and mannose metabolism; map00051

Carbohydrate Metabolism; Pentose phosphate pathway; map00030

Carbohydrate Metabolism; Glycolysis / Gluconeogenesis; map00010

Energy Metabolism; Carbon fixation; map00710

Organisms

Human (9606)

Genome localization

9q22.3[8789 ], 9q22.3[2203 ],

Comments

The animal enzyme also acts on sedoheptulose 1,7-bisphosphate.

Rate-limiting Description

"Although FBP is a rate-limiting enzyme in gluconeogenesis, using both parametric and nonparametric analysis there was no evidence for linkage of FBP to diabetes in these families." (8530070)

Regulatory Information

Regulatory type

Detail

key enzyme;

"Fructose-1,6-bisphosphatase (EC 3.1.3.11) is a key regulatory enzyme of gluconeogenesis that catalyzes the hydrolysis of fructose-1,6-bisphosphate to generate fructose-6-phosphate and inorganic phosphate." (7558035)

regulatory enzyme;

"Recently, the gene coding fructose 1,6 bisphosphatase (F1,6BP), a regulatory enzyme of gluconeogenesis, has been identified in type II cells and its promoter bears a Vitamin D response element." (7558035)

phosphorylation;

"A common phosphorylation site for cyclic AMP-dependent protein kinase and protein kinase C in human placental 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase." (9836440)

phosphorylation;

O00757

phosphorylation;

P09467

phosphorylation;

O00757:from_uniprot:216_Phosphotyrosine

Gene ontology

Gene ontology

GO:0008270 (F) zinc ion binding [P09467 ];
GO:0004331 (F) fructose-2, 6-bisphosphate 2-phosphatase act... [P09467, O00757 ];
GO:0042802 (F) identical protein binding [P09467 ];
GO:0006094 (P) gluconeogenesis [P09467, O00757 ];
GO:0005739 (C) mitochondrion [P09467 ];
GO:0042132 (F) fructose 1, 6-bisphosphate 1-phosphatase act... [P09467, O00757 ];
GO:0005829 (C) cytosol [P09467, O00757 ];
GO:0006000 (P) fructose metabolic process [P09467, O00757 ];

Disease relevance

Disease

Defects in FBP1 are the cause of fructose-1, 6-bisphosphatase deficiency (FBPD) [MIM:229700]. FBPD is 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 [P09467 ];

Links

SwissProt

O00757; P09467

Entrez Gene

2203; 8789

HPRD

04322; 01973



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  Last Modified: 2009-03-24  
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