succinate-CoA ligase GDP/ADP-forming subunit alpha

Normal function

The SUCLG1 gene provides instructions for making one part (the alpha subunit) of an enzyme called succinyl-CoA ligase. The alpha subunit is used to make two slightly different versions of this enzyme. One version is most active in tissues such as the brain and muscle that require a large amount of energy, while the other version is most active in tissues that require less energy.

Both versions of succinyl-CoA ligase play an important role in mitochondria, which are the energy-producing centers inside the cell. These enzymes are involved in a series of chemical reactions known as the citric acid cycle (or Krebs cycle). These reactions allow cells to use oxygen and produce energy.

Mitochondria each contain a small amount of DNA, known as mitochondrial DNA or mtDNA. Studies suggest that succinyl-CoA ligase interacts with another enzyme, called nucleoside diphosphate kinase, to produce and maintain the molecules that make up mtDNA. Having an adequate amount of mtDNA is essential for normal energy production within cells.

Conditions referring to this gene or chromosome

• Leigh syndrome

• SUCLG1-related mitochondrial DNA depletion syndrome

Other names

GALPHA

succinate-CoA ligase, ADP-forming, alpha subunit

succinate-CoA ligase, GDP-forming, alpha subunit

succinyl-CoA synthetase, alpha subunit

SUCLA1