SUCLG1

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.

Full Name

Succinate-CoA Ligase Alpha Subunit

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.

Biological Process

Biological Process succinyl-CoA catabolic process1 PublicationIC:ComplexPortal
Biological Process tricarboxylic acid cycleManual Assertion Based On ExperimentIBA:GO_Central

Cellular Location

Mitochondrion

Involvement in disease

Mitochondrial DNA depletion syndrome 9 (MTDPS9):
A severe disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, lactic acidosis, severe psychomotor retardation, progressive neurologic deterioration, and excretion of methylmalonic acid.