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]