SUCLG1
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.
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.
None
The disease is caused by variants affecting the gene represented in this entry.
Pathway
Carbohydrate metabolism; tricarboxylic acid cycle; succinate from succinyl-CoA (ligase route): step 1/1.
Sequence Similarities
Belongs to the succinate/malate CoA ligase alpha subunit family.
Cellular localization
- Mitochondrion
Alternative names
Succinyl-CoA synthetase subunit alpha, SCS-alpha, SUCLG1