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

Basic Information

Enzyme Number

2.7.1.11

Official Name

6-phosphofructokinase

Name from literature

phosphofructokinase

Pathway from literature

glycolysis

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

Carbohydrate Metabolism; Galactose metabolism; map00052

Organisms

Human (9606)

Genome localization

10p15.3-p15.2[5214 ], 12q13.3[5213 ], 21q22.3[5211 ],

Comments

D-Tagatose 6-phosphate and sedoheptulose 7-phosphate can act as acceptors. UTP, CTP and ITP can act as donors. Not identical with EC 2.7.1.105 6-phosphofructo-2-kinase.

Rate-limiting Description

"Time-response studies with Ca(2+)-ionophore A23187 have revealed dual effects on the distribution of phosphofructokinase (PFK) (EC 2.7.1.11), the rate-limiting enzyme of glycolysis, between the cytoskeletal and cytosolic (soluble) fractions of the cell." (10444344)

"We show that taxol affects both levels of regulation of glycolysis in melanoma cells; it decreases the levels of glucose 1,6-bisphosphate and fructose 1,6-bisphosphate, the two allosteric stimulatory signal molecules of glycolysis, and also causes a detachment of phosphofructokinase (ATP: D-fructose-6-phosphate 1-phosphotransferase, EC 2.7.1.11), the rate-limiting enzyme of glycolysis, from the cytoskeleton of B16 melanoma cells." (10323269)

"We studied muscle histology and measured the activities of the rate-limiting enzymes of anaerobic glycolysis (phosphofructokinase, PFK), glycogenolysis (phosphorylase), citric acid cycle (alpha-ketoglurate dehydrogenase, KGDH) and fatty acid oxidation (carnitinepalmitoyl transferase I and II, CPT I and II) from biopsies of the vastus lateralis muscle at baseline and after 3 and 6 months." (10742704)

Regulatory Information

Upstream transcription factor

6667;6670

Regulatory type

Detail

regulatory enzyme;

"Muscle 6-phospho-1-kinase (PFK) is the key regulatory enzyme of the glycolytic pathway and is a calmodulin-binding protein binding two calmodulin molecules per PFK protomer." (16377227#12023862#8880699)

phosphorylation;

"Covalent modification of phosphofructokinase by phosphorylation--dephosphorylation." (6276080#9836440)

phosphorylation;

P08237

phosphorylation;

P17858

phosphorylation;

Q01813

phosphorylation;

P08237:from_uniprot:2_Phosphothreonine

phosphorylation;

P08237:from_uniprot:775_Phosphoserine

phosphorylation;

P17858:from_uniprot:633_Phosphotyrosine

phosphorylation;

P17858:from_uniprot:640_Phosphotyrosine

phosphorylation;

Q01813:from_uniprot:386_Phosphoserine

phosphorylation;

Q01813:from_uniprot:651_Phosphotyrosine

phosphorylation;

Q01813:from_uniprot:783_Phosphoserine

transcriptional factor;Sp1(6667)

"Novel clustering of Sp1 transcription factor binding sites at the transcription initiation site of the human muscle phosphofructokinase P1 promoter." (7800504#8024691#1833270)

transcriptional factor;Sp1(6667),Sp3(6670),NF-Y

"Sp1 and Sp3 were shown to bind to most if not all of them. Additionally, an NF-Y binding site was identified. Results of deletion and mutation analysis suggest that all of these transcription factors contribute positively to promoter activity." (15716112)

Gene ontology

Gene ontology

GO:0000287 (F) magnesium ion binding [P17858, Q01813, P08237 ];
GO:0005945 (C) 6-phosphofructokinase complex [P17858, Q01813, P08237 ];
GO:0005783 (C) endoplasmic reticulum [P08237 ];
GO:0008022 (F) protein C-terminus binding [P08237 ];
GO:0006096 (P) glycolysis [P17858, Q01813, P08237 ];
GO:0070061 (F) fructose binding [P17858, P08237 ];
GO:0005977 (P) glycogen metabolic process [P08237 ];
GO:0005524 (F) ATP binding [P17858, Q01813, P08237 ];
GO:0042802 (F) identical protein binding [P17858, P08237 ];
GO:0019900 (F) kinase binding [P17858, P08237 ];
GO:0003872 (F) 6-phosphofructokinase activity [P17858, Q01813, Q16184, P08237 ];
GO:0051259 (P) protein oligomerization [P08237 ];
GO:0046716 (P) muscle maintenance [P08237 ];
GO:0006002 (P) fructose 6-phosphate metabolic process [P17858, P08237 ];
GO:0005515 (F) protein binding [Q01813 ];

Disease relevance

Disease

Defects in PFKM are the cause of glycogen storage disease type 7 (GSD7) [MIM:232800];
also known as Tarui disease. GSD7 is an autosomal recessive disorder characterized by exercise intolerance with associated nausea and vomiting. Short bursts of intense activity are particularly difficult. Severe muscle cramps and myoglobinuria develop after vigorous exercise. Most patients obtain a "second wind" when the onset of exercise is followed by a brief rest period. In time patients adjust their activity level and are well compensated [P08237 ];

Links

SwissProt

P08237; P17858; Q01813; Q16184

Entrez Gene

5211; 5213; 5214

HPRD

01385; 01384; 01988



  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min