Entrez gene ID | | 8803 |
Official gene symbol | | SUCLA2 |
Full name | | succinate-CoA ligase, ADP-forming, beta subunit |
Aliases | | ,A-BETA,SCS-betaA, |
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] |
Location | | Chromosome: 13 Locus: 13q12.2-q13.3 |
Gene position | | 48575462 - 48516791 Map Viewer |
Gene orientation | | minus |
Gene size | | 58672 bp |
Gene sequence |
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OMIM ID | | 603921 |
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