Protein Name:
Fumarate hydratase, mitochondrial
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UniprotKB/SwissProt ID: P07954 (P07954)
Gene Name:
FH
Organism: Homo sapiens (Human)
Function: Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (PubMed:30761759). Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable) 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 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) (PubMed:20231875, PubMed:26237645). 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) (PubMed:26237645)
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion. Cytoplasm, cytosol. Nucleus. Chromosome
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Graphical Visualization of S-nitrosylation Sites:
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The S-nitrosylation sites of P07954
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| No. |
Position |
S-nitrosylated Peptide |
Secondary Structure of S-nitrosylated Peptide |
Solvent Accessibility of nitrosylated Site |
PubMed ID |
| 1 |
333 |
ELSGAMNTTA C SLMKIANDIR |
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| 2 |
434 |
GDASVSFTEN C VVGIQANTER |
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