Protein
Fumarate hydratase, mitochondrial
Function
Catalyzes the reversible stereospecific interconversion of fumarate to L-malate. 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. 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.
Similarity
Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
Sequence
MLRASATRFLSQAKNMNNSPRLFSSASAALQKFRAERDTFGDLQVPADRYWGAQTQRSLQNFDIGGPTERMPEPLIRAFGVLKKAAATVNMTYGLDPKVGEAIQKAADEVIDGSLIDHFPLVVWQTGSGTQTKMNVNEVISNRAIELLGGELGSKAPVHPNDHVNMSQSSNDTFPTAMHVAAVVEIHGRLIPALTTLRDALQAKSAEFEHIIKIGRTHLQDATPLTLGQEFSGYTQQLTYGIARVQGTLERLYNLAQGGTAVGTGLNTRKGFDAKVAEAIASITGLPFKTAPNKFEALAAHDALVEAHGALNTVACSLMKIANDIRYLGSGPRCGLGELSLPENEPGSSIMPGKVNPTQCEAMTMVCAQVMGNNTAISVAGSNGQFELNVFKPVMIKNLIQSIRLISDASISFTKNCVVGIEANEKKISSIMNESLMLVTALNPHIGYDKAAKCAKKAHKEGTTLKEAALSLGYLTSEEFDQWVRPEDMISAKD