| 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] |
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