9swj
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Human ADP-forming succinyl-CoA ligase complex SUCLG1-SUCLA2 bound to coenzyme A
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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] Contents | ||||||||||||||||||||
This page was last modified 09:24, 22 October 2025.