Aliases for SUCLA2 Gene
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 succinate-coa ligase deficiency and mitochondrial dna depletion syndrome, encephalomyopathic form. Among its related pathways are Metabolism and Carbon metabolism. 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.