SUCLG1, Official Symbol SUCLG1provided by HGNC Official Full Name succinate-CoA ligase GDP/ADP-forming subunit alphaprovided by HGNC Primary source HGNC:HGNC:11449 See related Ensembl:ENSG00000163541 MIM:611224; AllianceGenome:HGNC:11449 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 GALPHA; MTDPS9; SUCLA1 Summary 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. [provided by RefSeq, Feb 2010] Expression Broad expression in kidney (RPKM 188.3), duodenum (RPKM 98.6) and 25 other tissues See more Orthologs mouse all