SUCLG1/G2 cleaves succinyl-CoA
GDP + Pi + SUCC-CoA → GTP + SUCCA + CoA-SH
- Reactome
- R-HSA-71775
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:22122
Inputs
- GDPCHEBI:58189, C10H12N5O11P2, mitochondrial matrix
- PiCHEBI:43474, HO4P, mitochondrial matrix
- SUCC-CoACHEBI:57292, C25H35N7O19P3S, mitochondrial matrix
Outputs
- GTPCHEBI:37565, C10H12N5O14P3, mitochondrial matrix
- SUCCACHEBI:30031, C4H4O4, mitochondrial matrix
- CoA-SHCHEBI:57287, C21H32N7O16P3S, mitochondrial matrix
Catalysed by
- succinate-CoA ligase (GDP-forming) activity of SUCLG1:SUCLG2 [mitochondrial matrix]EC 6.2.1.4
Literature
- Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is disrupted in sideroblastic anemia J Clin Invest, 2000
- Genetic evidence for the expression of ATP- and GTP-specific succinyl-CoA synthetases in multicellular eucaryotes J Biol Chem, 1998
- Characterization of the ATP- and GTP-specific succinyl-CoA synthetases in pigeon. The enzymes incorporate the same alpha-subunit. J Biol Chem, 1998
- Expression of two succinyl-CoA synthetases with different nucleotide specificities in mammalian tissues J Biol Chem, 2004
- Deficiency of the alpha subunit of succinate-coenzyme A ligase causes fatal infantile lactic acidosis with mitochondrial DNA depletion Am J Hum Genet, 2007
- Guanosine triphosphate, the primary product of phosphorylation coupled to the breakdown of succinyl coenzyme A Biochim Biophys Acta, 1954