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

Basic Information

Enzyme Number

4.1.1.32

Official Name

phosphoenolpyruvate carboxykinase (GTP)

Name from literature

phosphoenolpyruvate carboxykinase (GTP)

Pathway from literature

Glyceroneogenesis

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

Organisms

Human (9606)

Genome localization

14q12[5106 ], 20q13.31[5105 ],

Comments

ITP can act as phosphate donor.

Rate-limiting Description

"The rate-limiting enzyme in this pathway is the cytosolic form of phosphoenolpyruvate carboxykinase (GTP) (4.1.1.32) (PEPCK-C)." (14739071)

Regulatory Information

Upstream transcription factor

6667;6720;1051;1050

Regulatory type

Detail

key enzyme;

"Glyceroneogenesis is an important metabolic pathway for fatty acid reesterification in adipose tissue, thereby reducing fatty acid release. Glyceroneogenesis and cytosolic phosphoenolpyruvate carboxykinase (PEPCK-C), which is the key enzyme in this pathway, are both regulated by a series of hormones and nutrients, among which all-trans retinoic acid (all-trans RA) is a transcriptional inducer of the PEPCK-C gene (Pck1). " (18492826)

transcriptional factor;"C/EBPalpha(1050),C/EBPbeta(1051)"

"Conserved amino acids within CCAAT enhancer-binding proteins (C/EBP(alpha) and beta) regulate gene expression" (11997389)

transcriptional factor;"SREBP-1c(6720),Sp1(6667)"

"transcription of the gene for phosphoenolpyruvate carboxykinase (GTP) in the liver is regulated by SREBP-1c and Sp1" (14744869)

phosphorylation;

P35558

phosphorylation;

Q16822

phosphorylation;

Q16822:from_uniprot:593_Phosphothreonine

Gene ontology

Gene ontology

GO:0030145 (F) manganese ion binding [Q16822, P35558 ];
GO:0000287 (F) magnesium ion binding [P35558 ];
GO:0006094 (P) gluconeogenesis [Q16822, P35558 ];
GO:0005739 (C) mitochondrion [Q16822 ];
GO:0005525 (F) GTP binding [Q16822, P35558 ];
GO:0031406 (F) carboxylic acid binding [P35558 ];
GO:0046327 (P) glycerol biosynthetic process from pyruvate [P35558 ];
GO:0005829 (C) cytosol [P35558 ];
GO:0005813 (C) centrosome [P35558 ];
GO:0016301 (F) kinase activity [Q712L8 ];
GO:0004613 (F) phosphoenolpyruvate carboxykinase (GTP) act... [Q712L8, Q16822, P35558 ];
GO:0032868 (P) response to insulin stimulus [P35558 ];
GO:0005634 (C) nucleus [P35558 ];
GO:0042593 (P) glucose homeostasis [P35558 ];

Tissue expression

Tissue From HPRD

Small intestine [02026, 02028 ];
Kidney [02026, 02028 ];
Heart [02026 ];
Lung [02026 ];
Pancreas [02026 ];
Liver [02026, 02028 ];
Placenta [02026 ];
Brain [02026 ];

Tissue specificity

Major sites of expression are liver, kidney and adipocytes [P35558 ];

Subcellular localization

Localization

mitochondrion [Q16822 ];

cytoplasm [P35558 ];

Disease relevance

Disease

Defects in PCK2 are the cause of mitochondrial phosphoenolpyruvate carboxykinase deficiency (mitochondrial PEPCK deficiency) [MIM:261650]. PEPCK deficiency is a metabolic disorder resulting from impaired gluconeogenesis. It is a rare disease with less than 10 cases reported in the literature. Clinical characteristics include hypotonia, hepatomegaly, failure to thrive, lactic acidosis and hypoglycaemia. Autoposy reveals fatty infiltration of both the liver and kidneys. The disorder is transmitted as an autosomal recessive trait [Q16822 ];

Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (cytosolic PEPCK deficiency) [MIM:261680]. PEPCK deficiency is a metabolic disorder resulting from impaired gluconeogenesis. It is a rare disease with less than 10 cases reported in the literature. Clinical characteristics include hypotonia, hepatomegaly, failure to thrive, lactic acidosis and hypoglycaemia. Autoposy reveals fatty infiltration of both the liver and kidneys. The disorder is transmitted as an autosomal recessive trait [P35558 ];

Links

SwissProt

P35558; Q16822; Q712L8

Entrez Gene

5105; 5106

HPRD

02026; 02028



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