Recombinant Human Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial (SUCLG1)

Uniprot Id

P53597

Target Species

Human

Target Name

SUCLG1

Target Full Name

Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial

Target Function

Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits.

Target Involvement

Mitochondrial DNA depletion syndrome 9 (MTDPS9)

Target Subcellular Location

Mitochondrion.

Target Protein Families

Succinate/malate CoA ligase alpha subunit family

Target Synonyms

FLJ21114; FLJ43513; GALPHA; MTDPS9; SCS alpha; SCS-alpha; SUCA_HUMAN; Succinate CoA ligase alpha subunit ; Succinyl CoA ligase [GDP forming] subunit alpha mitochondrial; Succinyl CoA synthetase subunit alpha; Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha; mitochondrial; Succinyl-CoA synthetase subunit alpha; SUCLA1; SUCLG 1; SUCLG1

Target Background

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.