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Proteins/UNIPROT:P07954
Catalyzes the reversible stereospecific interconversion of fumarate to L-malate. Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable)..
[Isoform Mitochondrial]: Catalyzes the hydration of fumarate to L-malate in the tricarboxylic acid (TCA) cycle to facilitate a transition step in the production of energy in the form of NADH..
[Isoform Cytoplasmic]: Catalyzes the dehydration of L-malate to fumarate (By similarity). Fumarate metabolism in the cytosol plays a role during urea cycle and arginine metabolism; fumarate being a by-product of the urea cycle and amino-acid catabolism (By similarity). Also plays a role in DNA repair by promoting non-homologous end-joining (NHEJ). In response to DNA damage and phosphorylation by PRKDC, translocates to the nucleus and accumulates at DNA double-strand breaks (DSBs): acts by catalyzing formation of fumarate, an inhibitor of KDM2B histone demethylase activity, resulting in enhanced dimethylation of histone H3 'Lys-36' (H3K36me2)..
Function from UniProtKB (P07954), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
5 rows
131 statements
131 statements · 8 reference databases · Subject: FH · Sources: Bgee, gene ontology, Hetionet, Human Protein Atlas, Human-GEM, Reactome, Rhea, WikiPathways
3 reactions
Catalysed by FH
Reaction pageReversibleBalancedEC 4.2.1.2cytosol
Human-GEM MAR04408CC BY 4.0Reference data
Catalysed by FH
Reaction pageReversibleBalancedEC 4.2.1.2mitochondrial matrix
Human-GEM MAR04410CC BY 4.0Reference data
2 names
Resolves to: FH
Catalysed by FH
Reaction pageReversibleBalanced
Rhea RHEA:12460CC BY 4.0Reference data