InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: SUCLA2

Summary

InnateDB Protein

IDBP-31232.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

SUCLA2

Protein Name

succinate-CoA ligase, ADP-forming, beta subunit

Synonyms

A-BETA; MTDPS5; SCS-betaA;

Species

Homo sapiens

Ensembl Protein

ENSP00000367923

InnateDB Gene

IDBG-31230 (SUCLA2)

Protein Structure

UniProt Annotation

Function

Catalyzes the ATP-dependent ligation of succinate and CoA to form succinyl-CoA. {ECO:0000250}.

Subcellular Localization

Mitochondrion.

Disease Associations

Mitochondrial DNA depletion syndrome 5 (MTDPS5) [MIM:612073]: A disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, neurologic deterioration, a hyperkinetic-dystonic movement disorder, external ophthalmoplegia, deafness, variable renal tubular dysfunction, and mild methylmalonic aciduria in some patients. {ECO:0000269PubMed:15877282, ECO:0000269PubMed:17287286, ECO:0000269PubMed:17301081, ECO:0000269PubMed:23759946}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Widely expressed. Not expressed in liver and lung. {ECO:0000269PubMed:9765291}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 25 experimentally validated interaction(s) in this database.

Experimentally validated
Total	25 [view]
Protein-Protein	23 [view]
Protein-DNA	2 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003824	catalytic activity
GO:0004775	succinate-CoA ligase (ADP-forming) activity
GO:0005515	protein binding
GO:0005524	ATP binding
GO:0046872	metal ion binding

Biological Process

GO:0006099	tricarboxylic acid cycle
GO:0006104	succinyl-CoA metabolic process
GO:0006105	succinate metabolic process
GO:0006781	succinyl-CoA pathway
GO:0008152	metabolic process
GO:0044237	cellular metabolic process
GO:0044281	small molecule metabolic process

Cellular Component

GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix
GO:0070062	extracellular vesicular exosome

Protein Structure and Domains

PDB ID

InterPro

IPR005809 Succinyl-CoA synthetase, beta subunit
IPR005811 ATP-citrate lyase/succinyl-CoA ligase
IPR013650 ATP-grasp fold, succinyl-CoA synthetase-type
IPR016102 Succinyl-CoA synthetase-like

PFAM

PF00549
PF08442

PRINTS

PIRSF

PIRSF001554

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q9P2R7