Full name: succinate-CoA ligase ADP-forming subunit beta

Gene type: protein coding

RefSeq status: REVIEWED

Also known as: A-BETA; A-SCS; LINC00444; MTDPS5; SCS-betaA

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]

Expression: Ubiquitous expression in heart (RPKM 24.1), fat (RPKM 23.0) and 25 other tissues

Gene size: 58618bp

Exon count: 11