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Phosphorylated human E105Qa GTP-specific succinyl-CoA synthetase complexed with coenzyme APhosphorylated human E105Qa GTP-specific succinyl-CoA synthetase complexed with coenzyme A
Structural highlights
DiseaseSUCA_HUMAN Fatal infantile lactic acidosis with methylmalonic aciduria. The disease is caused by mutations affecting the gene represented in this entry. FunctionSUCA_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] |
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