FH tetramer hydrates fumarate to L-malate
FUMA + H2O → MAL
- Reactome
- R-HSA-70982
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:12462, RHEA:12460
Inputs
- FUMACHEBI:29806, C4H2O4, mitochondrial matrix
- H2OCHEBI:15377, H2O, mitochondrial matrix
Outputs
- MALCHEBI:15589, C4H4O5, mitochondrial matrix
Catalysed by
- fumarate hydratase activity of FH tetramer [mitochondrial matrix]EC 4.2.1.2
Literature
- Mutation of the fumarase gene in two siblings with progressive encephalopathy and fumarase deficiency J Clin Invest, 1994
- Structural, biochemical and biophysical characterization of recombinant human fumarate hydratase FEBS J, 2019
- The Pleiotropic Effects of Fumarate: From Mitochondrial Respiration to Epigenetic Rewiring and DNA Repair Mechanisms Metabolites, 2023
- Post-translational Modifications of Fumarase Regulate its Enzyme Activity and Function in Respiration and the DNA Damage Response J Mol Biol, 2020