SUCLA2

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]

Description

succinate-CoA ligase, ADP-forming, beta subunit

Protein family

Enzymes

Chromosome

13:q14.2 (48510622 - 48612125)

Ensembl ID

ENSG00000136143  (ver. 69.37)

Orthologs

Mouse Sucla2: ENSMUSG00000022110

Human splice variants
Mouse splice variants