Aliases for SUCLA2 Gene
- Succinate-CoA Ligase ADP-Forming Beta Subunit 2 3 5
- ATP-Specific Succinyl-CoA Synthetase Subunit Beta 3 4
- Succinyl-CoA Synthetase Beta-A Chain 3 4
- Succinate--CoA Ligase (ADP-Forming) 2 3
- Renal Carcinoma Antigen NY-REN-39 3 4
- EC 126.96.36.199 4 61
- SCS-BetaA 3 4
- Succinyl-CoA Ligase [ADP-Forming] Subunit Beta, Mitochondrial 3
External Ids for SUCLA2 Gene
Previous GeneCards Identifiers for SUCLA2 Gene
Succinyl-CoA synthetase (SCS) is a mitochondrial matrix enzyme that acts as a heterodimer, being composed of an invariant alpha subunit and a substrate-specific beta subunit. The protein encoded by this gene is an ATP-specific SCS beta subunit that dimerizes with the SCS alpha subunit to form SCS-A, an essential component of the tricarboxylic acid cycle. SCS-A hydrolyzes ATP to convert succinate to succinyl-CoA. Defects in this gene are a cause of myopathic mitochondrial DNA depletion syndrome. A pseudogene of this gene has been found on chromosome 6. [provided by RefSeq, Jul 2008]
GeneCards Summary for SUCLA2 Gene
SUCLA2 (Succinate-CoA Ligase ADP-Forming Beta Subunit) is a Protein Coding gene. Diseases associated with SUCLA2 include Mitochondrial Dna Depletion Syndrome 5 and Sucla2-Related Mitochondrial Dna Depletion Syndrome, Encephalomyopathic Form, With Mild Methylmalonic Aciduria. Among its related pathways are Pyruvate metabolism and Citric Acid (TCA) cycle and Valproic Acid Pathway, Pharmacodynamics. GO annotations related to this gene include succinate-CoA ligase (ADP-forming) activity. An important paralog of this gene is SUCLG2.
UniProtKB/Swiss-Prot for SUCLA2 Gene
Catalyzes the ATP-dependent ligation of succinate and CoA to form succinyl-CoA.