Aliases for SUCLA2 Gene
External Ids 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 sucla2-related mitochondrial dna depletion syndrome, encephalomyopathic form, with mild methylmalonic aciduria and mitochondrial dna depletion syndrome 5. Among its related pathways are Metabolism and 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.