Succinate–CoA ligase [GDP-forming] subunit beta, mitochondrial (SUCLG2)

SUCLG2 is the gene encoding the mitochondrial GTP-specific succinyl-CoA synthetase beta subunit, a key enzyme in the citric acid (TCA) cycle that catalyzes the conversion of succinyl-CoA and GDP (plus inorganic phosphate) to succinate, CoA, and GTP. This enzyme complex is essential for mediating substrate-level phosphorylation in mitochondria and for maintaining mitochondrial energy metabolism. Mutations in the gene are associated with rare mitochondrial depletion syndromes and related metabolic diseases, indicating its critical physiological role in cellular energy homeostasis.

Other names

GTP-specific succinyl-CoA synthetase beta subunitSuccinyl-CoA synthetase beta-G chainSuccinyl-CoA ligase GDP-forming beta subunitGTP-specific succinyl-CoA synthetase subunit betaSuccinyl-CoA ligase, GDP-forming, beta chain, mitochondrialSCS-betaGGBETA