SUCLA2, Official Symbol SUCLA2provided by HGNC Official Full Name succinate-CoA ligase ADP-forming subunit betaprovided by HGNC Primary source HGNC:HGNC:11448 See related Ensembl:ENSG00000136143 MIM:603921; AllianceGenome:HGNC:11448 Gene type protein coding RefSeq status REVIEWED Organism Homo sapiens Lineage Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo Also known as A-SCS; A-BETA; MTDPS5; LINC00444; 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 See more Orthologs mouse all