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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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FH tetramer hydrates fumarate to L-malate

FUMA + H2O → MAL

Reactome
R-HSA-70982
Compartment
mitochondrial matrix
Rhea
RHEA:12462, RHEA:12460

Inputs

Outputs

Catalysed by

  • fumarate hydratase activity of FH tetramer [mitochondrial matrix]EC 4.2.1.2

Literature

  1. Mutation of the fumarase gene in two siblings with progressive encephalopathy and fumarase deficiency J Clin Invest, 1994
  2. Structural, biochemical and biophysical characterization of recombinant human fumarate hydratase FEBS J, 2019
  3. The Pleiotropic Effects of Fumarate: From Mitochondrial Respiration to Epigenetic Rewiring and DNA Repair Mechanisms Metabolites, 2023
  4. Post-translational Modifications of Fumarase Regulate its Enzyme Activity and Function in Respiration and the DNA Damage Response J Mol Biol, 2020