Reactome | FH tetramer hydrates fumarate to L-malate

FH tetramer hydrates fumarate to L-malate

Stable Identifier

R-HSA-70982

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

fumarate + H2O => (S)-malate

ReviewStatus

5/5

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FH tetramer hydrates fumarate to L-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