UniProt ID: Q7SX99 |
FUNCTION: Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (By similarity). Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable). {ECO:0000250|UniProtKB:P07954, ECO:0000305}. FUNCTION: [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. {ECO:0000250|UniProtKB:P10173}. FUNCTION: [Isoform Cytoplasmic]: Catalyzes the dehydration of L-malate to fumarate. 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 translocates to the nucleus and accumulates at DNA double-strand breaks (DSBs): acts by catalyzing formation of fumarate (By similarity). {ECO:0000250|UniProtKB:P07954, ECO:0000250|UniProtKB:P97807}. CATALYTIC ACTIVITY: [Isoform Mitochondrial]: Reaction=(S)-malate = fumarate + H2O; Xref=Rhea:RHEA:12460, ChEBI:CHEBI:15377, ChEBI:CHEBI:15589, ChEBI:CHEBI:29806; EC=4.2.1.2; Evidence={ECO:0000250|UniProtKB:P10173}; PhysiologicalDirection=right-to-left; Xref=Rhea:RHEA:12462; Evidence={ECO:0000250|UniProtKB:P10173}; CATALYTIC ACTIVITY: [Isoform Cytoplasmic]: Reaction=(S)-malate = fumarate + H2O; Xref=Rhea:RHEA:12460, ChEBI:CHEBI:15377, ChEBI:CHEBI:15589, ChEBI:CHEBI:29806; EC=4.2.1.2; Evidence={ECO:0000250|UniProtKB:P97807}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:12461; Evidence={ECO:0000250|UniProtKB:P97807}; PATHWAY: Carbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1. {ECO:0000250|UniProtKB:P10173}. SUBUNIT: Homotetramer. {ECO:0000250|UniProtKB:P07954}. SUBCELLULAR LOCATION: [Isoform Mitochondrial]: Mitochondrion {ECO:0000250|UniProtKB:P07954}. SUBCELLULAR LOCATION: [Isoform Cytoplasmic]: Cytoplasm, cytosol {ECO:0000250|UniProtKB:P07954}. Nucleus {ECO:0000250|UniProtKB:P07954}. Chromosome {ECO:0000250|UniProtKB:P07954}. Note=Translocates to the nucleus in response to DNA damage: localizes to DNA double-strand breaks (DSBs). {ECO:0000250|UniProtKB:P07954}. MISCELLANEOUS: There are 2 substrate-binding sites: the catalytic A site, and the non-catalytic B site that may play a role in the transfer of substrate or product between the active site and the solvent. Alternatively, the B site may bind allosteric effectors. {ECO:0000250|UniProtKB:P05042, ECO:0000250|UniProtKB:P9WN93}. SIMILARITY: Belongs to the class-II fumarase/aspartase family. Fumarase subfamily. {ECO:0000305}. |
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