MDH2 dimer dehydrogenates MAL

Stable Identifier
Reaction [transition]
Homo sapiens
(S)-malate + NAD(+) => H(+) + NADH + oxaloacetate
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Mitochondrial malate dehydrogenase (MDH2) dimer catalyzes the reversible reaction of malate and NAD+ to form oxaloacetate (OA) and NADH + H+ (Luo et al. 2006; Eo et al., 2022). This reaction is highly endergonic but is pulled in the direction annotated here when the TCA cycle is operating. The active enzyme is a palmitoylated homodimer (Sanchez et al. 1998; Pei et al., 2022). Mutations in MDH2 can cause infantile epileptic encephalopathy (DEE51, MIM:617339; see Priestley et al., 2022).
Literature References
PubMed ID Title Journal Year
36420423 Malate dehydrogenase 2 deficiency is an emerging cause of pediatric epileptic encephalopathy with a recognizable biochemical signature

Aggarwal, A, Gowda, VK, Jolín García, PC, Hong, X, Srinivasan, VM, Pace, LM, Gropman, A, Eskandar, M, Trapane, P, Sen, K, Priestley, JRC, Engelhardt, NM, Lusk, L, Ganetzky, RD, Campbell, IM, Cuddapah, SR, Suwannarat, P, Helbig, I, Alves, CAPF

Mol Genet Metab Rep 2022
16740313 An NADH-tetrazolium-coupled sensitive assay for malate dehydrogenase in mitochondria and crude tissue homogenates

Liu, J, Wang, X, Long, J, Luo, C

J Biochem Biophys Methods 2006
9792106 Aggregation states of mitochondrial malate dehydrogenase

Hazlett, TL, Sanchez, SA, Jameson, DM, Brunet, JE

Protein Sci 1998
35366151 Palmitoylation of MDH2 by ZDHHC18 activates mitochondrial respiration and accelerates ovarian cancer growth

Li, KY, Fang, CY, Yang, HJ, Li, JT, Wen, W, Pei, X, Lu, HJ, Yin, M, Lei, QY, Qu, J, Lei, MZ, Shen, Y

Sci China Life Sci 2022
36139014 Structural Comparison of hMDH2 Complexed with Natural Substrates and Cofactors: The Importance of Phosphate Binding for Active Conformation and Catalysis

Ahn, HC, Eo, Y, Duong, MTH

Biomolecules 2022
Catalyst Activity

L-malate dehydrogenase activity of MDH2 dimer [mitochondrial matrix]

Orthologous Events
Cross References
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