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
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This page was last modified 13:58, 11 April 2018.