SCS-beta ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit. Belongs to the succinate/malate CoA ligase beta subunit family. ATP-specific subunit beta subfamily. Widely expressed. Not expressed in liver and lung. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - propanoate; EC; Ligase; Mitochondrial
Chromosomal Location of human Ortholog: 13q14.2
Cellular Component:  mitochondrial matrix; mitochondrion
Molecular Function:  ATP binding; magnesium ion binding; protein binding; succinate-CoA ligase (ADP-forming) activity
Biological Process:  succinate metabolic process; succinyl-CoA metabolic process; succinyl-CoA pathway; tricarboxylic acid cycle
Disease: Mitochondrial Dna Depletion Syndrome 5 (encephalomyopathic With Or Without Methylmalonic Aciduria)
Reference #:  Q9P2R7 (UniProtKB)
Alt. Names/Synonyms: A-BETA; A-SCS; ATP-specific succinyl-CoA synthetase subunit beta; ATP-specific succinyl-CoA synthetase, beta subunit; LINC00444; long intergenic non-protein coding RNA 444; mitochondrial succinyl-CoA ligase [ADP-forming] subunit beta; MTDPS5; Renal carcinoma antigen NY-REN-39; SCS-beta; SCS-betaA; SUCB1; Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial; succinate-CoA ligase ADP-forming beta subunit; succinate-CoA ligase ADP-forming subunit beta; succinate-CoA ligase beta subunit; succinate-CoA ligase, ADP-forming, beta subunit; Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial; Succinyl-CoA synthetase beta-A chain; SUCLA2
Gene Symbols: SUCLA2
Molecular weight: 50,317 Da
Basal Isoelectric point: 7.05  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene