FH tetramer hydrates fumarate to L-malate

Stable Identifier
Reaction [transition]
Homo sapiens
fumarate + H2O => (S)-malate
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Mitochondrial fumarate hydratase (FH) tetramer catalyzes the reversible reaction of fumarate and water to form malate, the seventh step of the TCA cycle (Ajalla Aleixo et al., 2019; Wang et al., 2020). Mutations in the FH gene can lead to fumarase deficiency (FMRD, MIM:606812) or to leiomyomatosis and renal cell cancer (HLRCC, MIM:150800) (Bourgeron et al., 1994; reviewed by Giallongo et al, 2023).
Literature References
PubMed ID Title Journal Year
33058874 Post-translational Modifications of Fumarase Regulate its Enzyme Activity and Function in Respiration and the DNA Damage Response

Lehming, N, Lim, TK, Lin, Q, Ramamurthy, D, Tan, J, Yip, J, Wang, S, Pines, O, Chua, A, Liu, J, Yu, Z

J Mol Biol 2020
8200987 Mutation of the fumarase gene in two siblings with progressive encephalopathy and fumarase deficiency

Chretien, D, Landrieu, P, Doonan, S, Bourgeron, T, Rabier, D, Poggi-Bach, J, Rötig, A, Letouzé, P, Munnich, A, Rustin, P

J Clin Invest 1994
37512586 The Pleiotropic Effects of Fumarate: From Mitochondrial Respiration to Epigenetic Rewiring and DNA Repair Mechanisms

Barbagallo, I, Tropea, E, Giallongo, S, Longhitano, L, Parenti, R, Li Volti, G, Ferrigno, J, Bramanti, V, Vicario, N, Costa, F, Giallongo, C, Tibullo, D

Metabolites 2023
30761759 Structural, biochemical and biophysical characterization of recombinant human fumarate hydratase

de Pádua, RAP, Nonato, MC, Rustiguel, JK, Rangel, VL, Ajalla Aleixo, MA

FEBS J 2019
Catalyst Activity

fumarate hydratase activity of FH tetramer [mitochondrial matrix]

Orthologous Events
Cross References
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