Fumarate hydratase, mitochondrial

UniProtKB accession:  P08417

Grouped By:  Matching UniProtKB accession

UniProtKB description:  Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (PubMed:3040736, PubMed:11585823, PubMed:1587456, PubMed:20231875). In mitochondrion, 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 (PubMed:1587456, PubMed:20231875). In cytoplasm and nucleus, involved in DNA repair in response to DNA damage: following DNA double-strand breaks (DSBs), translocates from the cytosol to the nucleus and promotes DNA repair by catalyzing the dehydration of L-malate to fumarate (PubMed:20231875).