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. 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. Also able to act as an ATP-specific itaconyl- and malyl-CoA synthetase.
Biological Context
Subcellular Location: Mitochondrion
Tissue Specificity: Widely expressed. Not expressed in liver and lung
Disease Association: 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. [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
Product Specifications
Recombinant Human Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2) is a recombinant protein from Homo sapiens (Human), expressed in E.coli, covering amino acids 53-463aa, with N-terminal 10xHis-tagged and C-terminal Myc-tagged tag, molecular weight 52.0kDa, purity Greater than 90% as determined by SDS-PAGE.. Suitable for ELISA and Western Blot applications. Explore more Enzyme proteins →
![Recombinant Human Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2), Homo sapiens (Human), E.coli, N-terminal 10xHis-tagged and C-terminal Myc-tagged, 52.0kDa, purity >90% as determined by SDS-PAGE.](https://www.biocrestsci.com/wp-content/uploads/protein/1/BCRECP-001716_SDS.jpg)