glutaryl-CoA + FAD => crotonyl-CoA + FADH2 + CO2
FAD + GL-CoA + 2 H+ → crotonyl-CoA + FADH2 + CO2
- Reactome
- R-HSA-71046
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:30848
Inputs
- FADCHEBI:57692, C27H30N9O15P2, mitochondrial matrix
- GL-CoACHEBI:57378, C26H37N7O19P3S, mitochondrial matrix
- 2 H+CHEBI:15378, H, mitochondrial matrix
Outputs
- crotonyl-CoACHEBI:57332, C25H36N7O17P3S, mitochondrial matrix
- FADH2CHEBI:58307, C27H33N9O15P2, mitochondrial matrix
- CO2CHEBI:16526, CO2, mitochondrial matrix
Catalysed by
- glutaryl-CoA dehydrogenase activity of GCDH tetramer [mitochondrial matrix]EC 1.3.8.6
Literature
- Crystal structures of human glutaryl-CoA dehydrogenase with and without an alternate substrate: structural bases of dehydrogenation and decarboxylation reactions Biochemistry, 2004
- Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH) Hum Mol Genet, 2008