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

Basic Information

Enzyme Number

Official Name

aconitate hydratase

Name from literature


Pathway from literature

Pathway from KEGG

Energy Metabolism; Reductive carboxylate cycle (CO2 fixation); map00720

Carbohydrate Metabolism; Glyoxylate and dicarboxylate metabolism; map00630

Carbohydrate Metabolism; Citrate cycle (TCA cycle); map00020


Human (9606)

Genome localization

22q11.2-q13.31|22q13.2|22q13.2-q13.31[50 ], 9p22-q32|9p21.1[48 ],


Besides interconverting citrate and cis-aconitate, it also interconverts cis-aconitate with isocitrate and, hence, interconverts citrate and isocitrate. The equilibrium mixture is 91% citrate, 6% isocitrate and 3% aconitate. cis-Aconitate is used to designate the isomer (Z)-prop-1-ene-1,2,3-tricarboxylate. An iron-sulfur protein, containing a [4Fe-4S] cluster to which the substrate binds.

Rate-limiting Description

"The high citrate to isocitrate ratio of about 33:1, compared to the expected value of about 10:1, supports suggestions that citrate to isocitrate oxidation by aconitase is a rate limiting step in prostatic citrate metabolism." (8115279)

Regulatory Information

Upstream transcription factor


Regulatory type


key enzyme;

"Mitochondrial aconitase (mACON) is the key enzyme for the citrate oxidation in the mitochondrial Krebs cycle." (16201454)


"Immunoprecipitation experiments demonstrated that aconitase was phosphorylated on serine residues." (17615160)

transcriptional factor;HBX(10542)

"Downregulated by HBX in human hepatoma cell lines." (18262302)

transcriptional factor;Sp1(6667)

"The m-aconitase promoter is contained in a 153-bp 5' fragment lacking a TATA or CAAT sequence. Sp1 binding to specific Sp1 site is needed for promoter activity. Other transcription factors are recruited through protein-protein interactions." (16598741)





Gene ontology

Gene ontology

GO:0005783 (C) endoplasmic reticulum [P21399 ];
GO:0051539 (F) 4 iron, 4 sulfur cluster binding [P21399, Q99798 ];
GO:0005829 (C) cytosol [P21399 ];
GO:0005506 (F) iron ion binding [P21399, Q99798 ];
GO:0003994 (F) aconitate hydratase activity [P21399, Q99798 ];
GO:0005794 (C) Golgi apparatus [P21399 ];
GO:0006099 (P) tricarboxylic acid cycle [P21399, Q99798 ];
GO:0005759 (C) mitochondrial matrix [Q99798 ];
GO:0005634 (C) nucleus [Q99798 ];
GO:0006101 (P) citrate metabolic process [P21399, Q99798 ];
GO:0003723 (F) RNA binding [P21399 ];

Subcellular localization


mitochondrion [Q99798 ];

cytoplasm [P21399 ];



P21399; Q99798

Entrez Gene

48; 50


00013; 00012

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