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

Basic Information

Enzyme Number

2.7.1.40

Official Name

pyruvate kinase

Name from literature

pyruvate kinase

Pathway from literature

glycolysis

Pathway from KEGG

Human Diseases; Metabolic Disorders; Type II diabetes mellitus; map04930

Cellular Processes; Endocrine System; Insulin signaling pathway; map04910

Carbohydrate Metabolism; Pyruvate metabolism; map00620

Nucleotide Metabolism; Purine metabolism; map00230

Carbohydrate Metabolism; Glycolysis / Gluconeogenesis; map00010

Energy Metabolism; Carbon fixation; map00710

Organisms

Human (9606)

Genome localization

15q22[5315 ], 1q21[5313 ],

Comments

UTP, GTP, CTP, ITP and dATP can also act as donors. Also phosphorylates hydroxylamine and fluoride in the presence of CO2.

Rate-limiting Description

"The effect of F-2,6-P2 on the rate-limiting enzymes of glycolysis, ie, hexokinase, phosphofructokinase (PFK), and pyruvate kinase, has also been examined." (2820531)

"We determined activity and protein expression of the three rate-limiting glycolytic enzymes phosphofructo kinase (PFK), pyruvate kinase (PK) and hexokinase (HXK) in a cytoplasmic fraction of homogenates of placentae obtained from IUGR and appropriate for gestational age (AGA) pregnancies." (15028426)

Regulatory Information

Upstream transcription factor

3172;6927

Regulatory type

Detail

key enzyme;

"By using inducible translocation trap, a reporter gene-based system to detect inducible nuclear translocation, we found that the M2 isoform of pyruvate kinase, a key enzyme in glycolysis, translocates into the nucleus by interleukin-3, but not by epidermal growth factor, stimulation." (17446165)

transcriptional factor;"HNF4alpha(3172),HNF1alpha(6927)"

"Hepatocyte nuclear factor (HNF) 1 and HNF4 are the main tissue-restricted transcription factors involved in tissue-specific expression of the L-PK gene" (8132632)

phosphorylation;

"phosphorylated at tyrosine residues in breast cancer patients; did not find any evidence that this phosphorylation is initiated by the oncoprotein HER-2/neu" (12820337)

phosphorylation;

P30613

phosphorylation;

P14618:from_uniprot:105_Phosphotyrosine

phosphorylation;

P14618:from_uniprot:148_Phosphotyrosine

phosphorylation;

P14618:from_uniprot:328_Phosphothreonine

phosphorylation;

P14618:from_uniprot:37_Phosphoserine

phosphorylation;

P14618:from_uniprot:390_Phosphotyrosine

phosphorylation;

P14618:from_uniprot:45_Phosphothreonine

phosphorylation;

P14618:from_uniprot:83_Phosphotyrosine

phosphorylation;

P30613:from_uniprot:43_Phosphoserine; by PKA

phosphorylation;

P30613:from_uniprot:556_Phosphothreonine

Gene ontology

Gene ontology

GO:0000287 (F) magnesium ion binding [Q16715, P30613, B1AVT1, Q16716, Q504U3, P14618, B1AVT2 ];
GO:0004743 (F) pyruvate kinase activity [P30613, Q16716, O75758, Q504U3, B1AVT2, Q16715, B1AVT1, P14618 ];
GO:0006096 (P) glycolysis [P30613, Q16716, O75758, Q504U3, B1AVT2, Q16715, B1AVT1, P14618 ];
GO:0030955 (F) potassium ion binding [Q16715, P30613, B1AVT1, Q16716, Q504U3, P14618, B1AVT2 ];
GO:0005829 (C) cytosol [P14618 ];
GO:0005515 (F) protein binding [P14618 ];

Disease relevance

Disease

Defects in PKLR are the cause of pyruvate kinase hyperactivity [MIM:102900];
also known as high red cell ATP syndrome. This autosomal dominant phenotype is characterized by increase of red blood cell ATP [P30613 ];

Defects in PKLR are a cause of chronic nonspherocytic hemolytic anemia (CNSHA) [MIM:266200];
also called hereditary nonspherocytic hemolytic anemia (HNSHA) [P30613 ];

Links

SwissProt

B1AVT1; B1AVT2; O75758; P14618; P30613; Q16715; Q16716; Q504U3

Entrez Gene

5313; 5315

HPRD

11841; 01529



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