Reactome | MDH2 dimer dehydrogenates MAL

MDH2 dimer dehydrogenates MAL

Stable Identifier

R-HSA-70979

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

(S)-malate + NAD(+) => H(+) + NADH + oxaloacetate

ReviewStatus

5/5

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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 Priestley, JRC, Pace, LM, Sen, K, Aggarwal, A, Alves, CAPF, Campbell, IM, Cuddapah, SR, Engelhardt, NM, Eskandar, M, Jolín García, PC, Gropman, A, Helbig, I, Hong, X, Gowda, VK, Lusk, L, Trapane, P, Srinivasan, VM, Suwannarat, P, Ganetzky, RD	Mol Genet Metab Rep	2022
16740313	An NADH-tetrazolium-coupled sensitive assay for malate dehydrogenase in mitochondria and crude tissue homogenates Luo, C, Wang, X, Long, J, Liu, J	J Biochem Biophys Methods	2006
9792106	Aggregation states of mitochondrial malate dehydrogenase Sanchez, SA, Hazlett, TL, Brunet, JE, Jameson, DM	Protein Sci	1998
35366151	Palmitoylation of MDH2 by ZDHHC18 activates mitochondrial respiration and accelerates ovarian cancer growth Pei, X, Li, KY, Shen, Y, Li, JT, Lei, MZ, Fang, CY, Lu, HJ, Yang, HJ, Wen, W, Yin, M, Qu, J, Lei, QY	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 Eo, Y, Duong, MTH, Ahn, HC	Biomolecules	2022