| Primary Identifier | IPR034723 | Type | Family |
| Short Name | Succ_CoA_betaA_euk |
| description | This entry includes the beta subunit of the mitochondrial, ATP-forming succinate--CoA ligase (SUCLA;) from eukaryotes. The ligase functions in the citric acid cycle (TCA), and is a heterodimer of an alpha and beta subunit. It couples the hydrolysis of succinyl-CoA to the synthesis of ATP which is the only substrate-level phosphorylation step in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sitesfor coenzyme A and phosphate are found in the alpha subunit. GTP-forming succinate--CoA ligase has the same alpha subunit but a different beta subunit []. Deficiency of SUCLA activity is associated with encephalomyopathy and mitochondrial DNA depletion []. |
