SUCLA2

Succinyl-CoA synthetase (SCS) is a mitochondrial matrix enzyme that acts as a heterodimer, being composed of an invariant alpha subunit and a substrate-specific beta subunit. The protein encoded by this gene is an ATP-specific SCS beta subunit that dimerizes with the SCS alpha subunit to form SCS-A, an essential component of the tricarboxylic acid cycle. SCS-A hydrolyzes ATP to convert succinate to succinyl-CoA. Defects in this gene are a cause of myopathic mitochondrial DNA depletion syndrome. A pseudogene of this gene has been found on chromosome 6.

Full Name

Succinate-CoA Ligase ADP-Forming Beta Subunit

Function

ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA (PubMed:15877282).
The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit (By similarity).

Biological Process

Biological Process succinate metabolic processIEA:Ensembl
Biological Process succinyl-CoA catabolic process1 PublicationIC:ComplexPortal
Biological Process succinyl-CoA metabolic processManual Assertion Based On ExperimentIBA:GO_Central
Biological Process succinyl-CoA pathway1 PublicationNAS:UniProtKB
Biological Process tricarboxylic acid cycleManual Assertion Based On ExperimentIBA:GO_Central

Cellular Location

Mitochondrion

Involvement in disease

Mitochondrial DNA depletion syndrome 5 (MTDPS5):
A disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, neurologic deterioration, a hyperkinetic-dystonic movement disorder, external ophthalmoplegia, deafness, variable renal tubular dysfunction, and mild methylmalonic aciduria in some patients.