Protein Name succinate-CoA ligase ADP-forming subunit beta
Organism Homo sapiens
Gene ID 8803
Gene Symbol

SUCLA2

UniProt Q9P2R7 (SUCB1_HUMAN), E5KS60 (E5KS60_HUMAN), Q9Y4T0 (Q9Y4T0_HUMAN)
Relationships Total Number of functionally related compound(s) : 59
Total Number of Articles : 49
Description

succinate-CoA ligase ADP-forming subunit beta

Gene Summary

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]

synonyms
  • succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial
  • ATP-specific succinyl-CoA synthetase subunit beta
  • ATP-specific succinyl-CoA synthetase, beta subunit
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Properties