6g4q

Structural highlights

Disease

[SUCA_HUMAN] Fatal infantile lactic acidosis with methylmalonic aciduria. The disease is caused by mutations affecting the gene represented in this entry. [SUCB1_HUMAN] Mitochondrial DNA depletion syndrome, encephalomyopathic form with methylmalonic aciduria. The disease is caused by mutations affecting the gene represented in this entry.

Function

[SUCA_HUMAN] Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits.[HAMAP-Rule:MF_03222] [SUCB1_HUMAN] 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).[HAMAP-Rule:MF_03220]^[1]

References

1. ↑ Elpeleg O, Miller C, Hershkovitz E, Bitner-Glindzicz M, Bondi-Rubinstein G, Rahman S, Pagnamenta A, Eshhar S, Saada A. Deficiency of the ADP-forming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion. Am J Hum Genet. 2005 Jun;76(6):1081-6. doi: 10.1086/430843. Epub 2005 Apr 22. PMID:15877282 doi:http://dx.doi.org/10.1086/430843