这个基因编码异二聚酶琥珀酸辅酶A连接酶的α亚单位。这种酶以线粒体为靶点，催化琥珀酰辅酶A和ADP或GDP转化为琥珀酸酯和ATP或GTP。该基因突变是代谢紊乱致死性婴儿乳酸酸中毒和线粒体dna缺失的原因。
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.