succinylCoAksi
Succinyl-CoA synthetase, also known as succinate thiokinase, is a mitochondrial enzyme of the citric acid cycle (Krebs cycle). It catalyzes the conversion of succinyl-CoA to succinate while transferring a phosphate group to a nucleotide triphosphate, yielding either GTP or ATP. The overall reaction is generally described as succinyl-CoA + Pi + GDP (or ADP) ⇌ succinate + CoA + GTP (or ATP), with the exact nucleotide produced depending on the enzyme isoform present in the organism or tissue.
In the mitochondrion, succinyl-CoA synthetase operates at one of the few steps in the cycle that generates
The enzyme exists as distinct heteromeric complexes encoded by different nuclear genes. In mammals, two main
Genetic defects in SUCLG1, SUCLA2, or SUCLG2 can lead to mitochondrial disease phenotypes, including hypotonia, developmental