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

Basic Information

Enzyme Number

6.2.1.5

Official Name

succinate---CoA ligase (ADP-forming)

Name from literature

ATP-dependent citrate lyase

Pathway from literature

the de novo synthesis of lipids and cholesterol

Pathway from KEGG

Carbohydrate Metabolism; C5-Branched dibasic acid metabolism; map00660

Carbohydrate Metabolism; Propanoate metabolism; map00640

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

Carbohydrate Metabolism; Citrate cycle (TCA cycle); map00020

Organisms

Human (9606)

Genome localization

13q12.2-q13.3[8803 ],

Rate-limiting Description

"The mode of action of these compounds in the 50-100 microM concentration range appeared to be by increasing lipid excretion from the body and by inhibiting rate-limiting enzyme activities for the de novo synthesis of lipids and cholesterol (e.g., phosphatidylate phosphohydrolase, ATP-dependent citrate lyase, cytoplasmic acetyl coenzyme A ." (CoA synthetase, HMG CoA reductase, and acetyl CoA carboxylase))

"These data indicate preferential localization of ATP-citrate lyase in cholinergic nerve endings, and indicate that this enzyme is not a rate limiting step in the synthesis of the acetyl moiety of ACh in brain.." (6109001)

Regulatory Information

Regulatory type

Detail

phosphorylation;

"Immunoaffinity profiling of tyrosine phosphorylation in cancer cells." (15592455)

phosphorylation;

Q9P2R7

phosphorylation;

Q9P2R7:from_uniprot:279_Phosphoserine

phosphorylation;

Q9P2R7:from_uniprot:84_Phosphotyrosine

Gene ontology

Gene ontology

GO:0006099 (P) tricarboxylic acid cycle [Q9P2R7 ];
GO:0006781 (P) succinyl-CoA pathway [Q9P2R7 ];
GO:0005739 (C) mitochondrion [Q9P2R7 ];
GO:0004775 (F) succinate-CoA ligase (ADP-forming) activity [Q9P2R7 ];
GO:0005515 (F) protein binding [Q9P2R7 ];
GO:0005524 (F) ATP binding [Q9P2R7 ];

Tissue expression

Tissue From HPRD

Thymus [06798 ];
Colon [06798 ];
Lymph node [06798 ];
Ubiquitous [06798 ];
Fetus [06798 ];
Leukocyte [06798 ];
Testis [06798 ];
Bone marrow [06798 ];
Skeletal muscle [06798 ];
Liver [06798 ];
Brain [06798 ];
Spleen [06798 ];
Prostate [06798 ];
Small intestine [06798 ];
Kidney [06798 ];
Heart [06798 ];
Ovary [06798 ];
Pancreas [06798 ];
Placenta [06798 ];

Tissue specificity

Widely expressed [Q9P2R7 ];

Subcellular localization

Localization

mitochondrion [Q9P2R7 ];

Disease relevance

Disease

Defects in SUCLA2 are the cause of encephalomyopathic mitochondrial DNA depletion syndrome with methylmalonic aciduria (EMDSMA) [MIM:612073]. Mitochondrial DNA depletion syndrome (MDS) is a clinically heterogeneous group of disorders characterized by a reduction in mitochondrial DNA (mtDNA) copy number. The encephalomyopathic form with methylmalonic aciduria is characterized by hypotonia, muscle atrophy, hyperkinesia, severe hearing impairment and postnatal growth retardation [Q9P2R7 ];

Links

SwissProt

Q9P2R7

Entrez Gene

8803

HPRD

06798



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