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This gene encodes the alpha subunit of the heterodimeric enzyme succinate coenzyme A ligase. This enzyme is targeted to the mitochondria and catalyzes the conversion of succinyl CoA and ADP or GDP to succinate and ATP or GTP. Mutations in this gene are the cause of the metabolic disorder fatal infantile lactic acidosis and mitochondrial DNA depletion.
1500000I01Rik; GALPHA; MTDPS9; SCS-alpha; Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; succinate-CoA ligase alpha subunit; succinate-CoA ligase, alpha subunit; succinate-CoA ligase, GDP-forming alpha subunit; succinate-CoA ligase, GDP-forming, alpha subunit; succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; succinyl-CoA ligase [GDP-forming] subunit alpha, mitochondrial; succinyl-CoA synthetase alpha subunit; succinyl-CoA synthetase subunit alpha; SUCLA1; Suclg1
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