Gene Symbol: Mdh2
Description: malate dehydrogenase 2
Alias: Mor1, malate dehydrogenase, mitochondrial, malate dehydrogenase 2, NAD (mitochondrial)
Species: rat
Products:     Mdh2

Top Publications

  1. Curti D, Marzatico F, Dagani F, Villa R, Benzi G. Effects of ischemia and pharmacological treatment on subcellular fractions from neonatal rat brain. Farmaco Sci. 1982;37:275-82 pubmed
    ..The i.p. treatment with trimetazidine (at the dose level of 50 mg . kg-1) was without any significant effect on the tested enzymatic activities. ..
  2. Luo C, Wang X, Long J, Liu J. An NADH-tetrazolium-coupled sensitive assay for malate dehydrogenase in mitochondria and crude tissue homogenates. J Biochem Biophys Methods. 2006;68:101-11 pubmed
    ..5 microg protein of isolated mitochondria or crude tissue homogenate. These results suggest that this assay is a simple, sensitive, stable and inexpensive method with wide application. ..
  3. Ying W. NAD+/NADH and NADP+/NADPH in cellular functions and cell death: regulation and biological consequences. Antioxid Redox Signal. 2008;10:179-206 pubmed
    ..Future investigation into the metabolism and biological functions of NAD and NADP may expose fundamental properties of life, and suggest new strategies for treating diseases and slowing the aging process. ..
  4. Grant P, Roderick S, Grant G, Banaszak L, Strauss A. Comparison of the precursor and mature forms of rat heart mitochondrial malate dehydrogenase. Biochemistry. 1987;26:128-34 pubmed
    ..Only 12/314 differences are found; most are conservative changes, and all are on or near the surface of the enzyme. We propose that the transit peptide is located on the surface of the mitochondrial malate dehydrogenase precursor. ..
  5. Zhao S, Xu W, Jiang W, Yu W, Lin Y, Zhang T, et al. Regulation of cellular metabolism by protein lysine acetylation. Science. 2010;327:1000-4 pubmed publisher
    ..Our study reveals that acetylation plays a major role in metabolic regulation. ..
  6. Gnanapragasam A, Yogeeta S, Subhashini R, Ebenezar K, Sathish V, Devaki T. Adriamycin induced myocardial failure in rats: protective role of Centella asiatica. Mol Cell Biochem. 2007;294:55-63 pubmed
    ..Moreover, on the basis of our findings it may be concluded that the aqueous extract of C. asiatica not only possesses antioxidant properties but it may also reduce the extent of mitochondrial damage. ..
  7. McKenna M, Waagepetersen H, Schousboe A, Sonnewald U. Neuronal and astrocytic shuttle mechanisms for cytosolic-mitochondrial transfer of reducing equivalents: current evidence and pharmacological tools. Biochem Pharmacol. 2006;71:399-407 pubmed
    ..This report evaluates the evidence for each shuttle in brain cells and the drugs that can be used as pharmacological tools to study these shuttles. ..
  8. D Souza S, Srere P. Cross-linking of mitochondrial matrix proteins in situ. Biochim Biophys Acta. 1983;724:40-51 pubmed
    ..These results are interpreted to mean that in liver mitochondria, the Krebs cycle enzyme are preferentially located near the membrane. ..
  9. Long J, Wang X, Gao H, Liu Z, Liu C, Miao M, et al. Malonaldehyde acts as a mitochondrial toxin: Inhibitory effects on respiratory function and enzyme activities in isolated rat liver mitochondria. Life Sci. 2006;79:1466-72 pubmed

More Information


  1. Wiseman M, McKay D, Crow K, Hardman M. Rat liver mitochondrial malate dehydrogenase: purification, kinetic properties, and role in ethanol metabolism. Arch Biochem Biophys. 1991;290:191-6 pubmed
  2. Grant P, Tellam J, May V, Strauss A. Isolation and nucleotide sequence of a cDNA clone encoding rat mitochondrial malate dehydrogenase. Nucleic Acids Res. 1986;14:6053-66 pubmed
    ..The amino acid composition of the transit peptide is polar and basic. The pre-mMDH transit peptide shows marked homology with those of two other enzymes targeted to the rat mitochondrial matrix. ..
  3. Ramos A, Ferreira G, Carvalho Silva M, Furlanetto C, Gonçalves C, Ferreira G, et al. Acute administration of l-tyrosine alters energetic metabolism of hippocampus and striatum of infant rats. Int J Dev Neurosci. 2013;31:303-7 pubmed publisher
    ..If the same effects occur in the brain of the patients, it is possible that energy metabolism impairment may be contribute to possible damage in memory and cognitive processes in patients with tyrosinemia type II. ..
  4. Tan C, Tuch B, Tu J, Brown S. Role of NADH shuttles in glucose-induced insulin secretion from fetal beta-cells. Diabetes. 2002;51:2989-96 pubmed
    ..We concluded that the immaturity of the NADH shuttles contributes to the inability of fetal beta-cells to secrete insulin in response to glucose. ..
  5. Mavrides C, Nadeau G. Purification and properties of the cytosolic and mitochondrial forms of aspartate aminotransferase and malate dehydrogenase from rat heart. Biochem Cell Biol. 1987;65:239-44 pubmed
    ..Michaelis constants and maximal velocities were derived by the use of primary and secondary plots. In general, the properties of the enzymes from rat heart are similar to the properties of the enzymes from other animal sources. ..
  6. Rzem R, Vincent M, Van Schaftingen E, Veiga da Cunha M. L-2-hydroxyglutaric aciduria, a defect of metabolite repair. J Inherit Metab Dis. 2007;30:681-9 pubmed
    ..The latter enzyme appears, therefore, to be responsible for a 'metabolite repair' phenomenon and to belong to the expanding class of 'house-cleaning' enzymes. ..
  7. Ait El Mkadem S, Dayem Quere M, Gusic M, Chaussenot A, Bannwarth S, François B, et al. Mutations in MDH2, Encoding a Krebs Cycle Enzyme, Cause Early-Onset Severe Encephalopathy. Am J Hum Genet. 2017;100:151-159 pubmed publisher
    b>MDH2 encodes mitochondrial malate dehydrogenase (MDH), which is essential for the conversion of malate to oxaloacetate as part of the proper functioning of the Krebs cycle...
  8. Chow K, Ma Z, Cai J, Pierce W, Hersh L. Nardilysin facilitates complex formation between mitochondrial malate dehydrogenase and citrate synthase. Biochim Biophys Acta. 2005;1723:292-301 pubmed
    ..CS was co-immunoprecipitated with mMDH only in the presence of nardilysin showing that nardilysin facilitates complex formation. ..
  9. Ithayarasi A, Shyamala Devi C. Effect of alpha-tocopherol on mitochondrial electron transport in experimental myocardial infarction in rats. Indian J Biochem Biophys. 1998;35:115-9 pubmed
    ..In alpha-tocopherol pretreated rats, the activities of TCA cycle enzymes, concentration of cytochromes and the oxidation of succinate in state 3 and state 4 were retained at near normal values, following isoproterenol administration. ..
  10. Muchi H, Yamamoto Y. Studies on mitochondrial and cytoplasmic malate dehydrogenase in childhood myelodysplastic syndrome. Blood. 1983;62:808-14 pubmed
    ..Both enzymes from leukemic cells of four patients with active disease revealed much higher activities than controls, an expression of partially enhanced oxidative phosphorylation. ..
  11. Shows T, Chapman V, Ruddle F. Mitochondrial malate dehydrogenase and malic enzyme: Mendelian inherited electrophoretic variants in the mouse. Biochem Genet. 1970;4:707-18 pubmed
  12. Mortensen M, Ferguson D, Edelmann M, Kessler B, Morten K, Komatsu M, et al. Loss of autophagy in erythroid cells leads to defective removal of mitochondria and severe anemia in vivo. Proc Natl Acad Sci U S A. 2010;107:832-7 pubmed publisher
    ..In summary, we show that the selective removal of mitochondria by autophagy, but not other organelles, during erythropoeisis is essential and that this is a necessary developmental step in erythroid cells...
  13. Lai J, Clark J. Preparation and properties of mitochondria derived from synaptosomes. Biochem J. 1976;154:423-32 pubmed
    ..5. The results are also discussed in the light of the suggested connection between the heterogeneity of brain mitochondria and metabolic compartmentation. ..