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

Basic Information

Enzyme Number

Official Name

phosphoenolpyruvate carboxykinase (GTP)

Name from literature

phosphoenolpyruvate carboxykinase (GTP)

Pathway from literature


Pathway from KEGG

Cellular Processes; Endocrine System; Insulin signaling pathway; map04910

Cellular Processes; Endocrine System; Adipocytokine signaling pathway; map04920

Carbohydrate Metabolism; Pyruvate metabolism; map00620

Cellular Processes; Endocrine System; PPAR signaling pathway; map03320

Carbohydrate Metabolism; Citrate cycle (TCA cycle); map00020


Mouse (10090)

Genome localization

14 C1|14 21.0 cM[74551 ], 2 C1-qter|2 103.0 cM[18534 ],


ITP can act as phosphate donor.

Rate-limiting Description

"Evidence from transgenic mice that glucokinase is rate limiting for glucose utilization in the liver." (8751724)

"This enzyme catalyzes the rate-limiting step in hepatic and renal gluconeogenesis and adipose glyceroneogenesis." (7799943)

"In transient assays, PGC-1 potentiated glucocorticoid induction of the gene for phosphoenolpyruvate carboxykinase (PEPCK), the rate-limiting enzyme in gluconeogenesis." (11557984)

Regulatory Information

Upstream transcription factor


Regulatory type




transcriptional factor;ERRalpha(26379)

"ERRalpha acts as a transcriptional repressor of the PEPCK gene" (16267049)

transcriptional factor;Nuclear receptor(RARE)

"Nuclear receptors bind to retinoic acid response elements (RAREs) in the PEPCK promoter in liver." (16317119)

transcriptional factor;tumor necrosis factor(21926)

"Identification of tumor necrosis factor as a transcriptional regulator of the phosphoenolpyruvate carboxykinase gene following endotoxin treatment of mice." (1398916)

transcriptional factor;"HNF-4alpha(15378),RXRalpha(20181),RARalpha(19401),PPARalpha(19013),chicken ovalbumin upstream promoter transcription factor II(11819)"

"HNF-4alpha, RXR alpha, RAR alpha, PPAR alpha & chicken ovalbumin upstream promoter transcription factor II bind to the downstream retinoic acid response unit RARE1/RARE2, & PPARalpha & RXRalpha bind to the upstream RARE3 of the PEPCK gene." (16713227)

transcriptional factor;Hepatic nuclear factor 3

"Hepatic nuclear factor 3- and hormone-regulated expression of the phosphoenolpyruvate carboxykinase and insulin-like growth factor-binding protein 1 genes." (7532283)

transcriptional factor;Foxo1(56458)

"Foxa2 regulates lipid metabolism and ketogenesis in the liver during fasting and in diabetes." (15616563)

Gene ontology

Gene ontology

GO:0030145 (F) manganese ion binding [Q8BH04 ];
GO:0004613 (F) phosphoenolpyruvate carboxykinase (GTP) act... [Q8BH04, Q9Z2V4 ];
GO:0006094 (P) gluconeogenesis [Q8BH04, Q9Z2V4 ];
GO:0005739 (C) mitochondrion [Q8BH04 ];
GO:0005525 (F) GTP binding [Q8BH04, Q9Z2V4 ];
GO:0046327 (P) glycerol biosynthetic process from pyruvate [Q9Z2V4 ];
GO:0005737 (C) cytoplasm [Q9Z2V4 ];

Subcellular localization


mitochondrion [Q8BH04 ];

cytoplasm [Q9Z2V4 ];



Q8BH04; Q9Z2V4

Entrez Gene

18534; 74551

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