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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.
Gene Name: | Succinate-CoA Ligase, ADP-forming, Beta Subunit |
Synonyms: | SUCLA2, A-BETA, MTDPS5, SCS-betaA |
Target Sequences: | NM_003850 NP_003841.1 Q9P2R7 |
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