dimethylglycine dehydrogenase

Summary

Summary: A FLAVOPROTEIN enzyme that catalyzes the oxidative demethylation of dimethylglycine to SARCOSINE and FORMALDEHYDE.

Top Publications

  1. Porter D, Cook R, Wagner C. Enzymatic properties of dimethylglycine dehydrogenase and sarcosine dehydrogenase from rat liver. Arch Biochem Biophys. 1985;243:396-407 pubmed
    b>Dimethylglycine dehydrogenase (EC 1.5.99.2) and sarcosine dehydrogenase (EC 1.5.99.1) are flavoproteins which catalyze the oxidative demethylation of dimethylglycine to sarcosine and sarcosine to glycine, respectively...
  2. Binzak B, Wevers R, Moolenaar S, Lee Y, Hwu W, Poggi Bach J, et al. Cloning of dimethylglycine dehydrogenase and a new human inborn error of metabolism, dimethylglycine dehydrogenase deficiency. Am J Hum Genet. 2001;68:839-47 pubmed
    b>Dimethylglycine dehydrogenase (DMGDH) (E.C. number 1.5.99.2) is a mitochondrial matrix enzyme involved in the metabolism of choline, converting dimethylglycine to sarcosine...
  3. Moolenaar S, Poggi Bach J, Engelke U, Corstiaensen J, Heerschap A, De Jong J, et al. Defect in dimethylglycine dehydrogenase, a new inborn error of metabolism: NMR spectroscopy study. Clin Chem. 1999;45:459-64 pubmed
    ..The high concentration of DMG was caused by a deficiency of the enzyme dimethylglycine dehydrogenase (DMGDH). A homozygous missense mutation was found in the DMGDH gene of the patient...
  4. McAndrew R, Vockley J, Kim J. Molecular basis of dimethylglycine dehydrogenase deficiency associated with pathogenic variant H109R. J Inherit Metab Dis. 2008;31:761-8 pubmed publisher
    b>Dimethylglycine dehydrogenase (DMGDH) is a mitochondrial matrix flavoprotein that catalyses the demethylation of dimethylglycine to form sarcosine, accompanied by the reduction of the covalently bound FAD cofactor...
  5. Cook R, Misono K, Wagner C. The amino acid sequences of the flavin-peptides of dimethylglycine dehydrogenase and sarcosine dehydrogenase from rat liver mitochondria. J Biol Chem. 1985;260:12998-3002 pubmed
    The flavoenzymes dimethylglycine dehydrogenase (EC 1.5.99.2) and sarcosine dehydrogenase (EC 1.5.99...
  6. Brizio C, Brandsch R, Bufano D, Pochini L, Indiveri C, Barile M. Over-expression in Escherichia coli, functional characterization and refolding of rat dimethylglycine dehydrogenase. Protein Expr Purif. 2004;37:434-42 pubmed
    b>Dimethylglycine dehydrogenase (Me(2)GlyDH) is a mitochondrial enzyme that catalyzes the oxidative demethylation of dimethylglycine to sarcosine...
  7. Lang H, Minaian K, Freudenberg N, Hoffmann R, Brandsch R. Tissue specificity of rat mitochondrial dimethylglycine dehydrogenase expression. Biochem J. 1994;299 ( Pt 2):393-8 pubmed
    Expression of mitochondrial dimethylglycine dehydrogenase (Me2GlyDH) was analysed in various tissues, liver cell types and developmental stages of the rat...
  8. Mostowska A, Hozyasz K, Biedziak B, Misiak J, Jagodzinski P. Polymorphisms located in the region containing BHMT and BHMT2 genes as maternal protective factors for orofacial clefts. Eur J Oral Sci. 2010;118:325-32 pubmed publisher
    ..betaine-homocysteine methyltransferase-2 (BHMT2), choline dehydrogenase (CHDH), choline kinase (CHKA), dimethylglycine dehydrogenase (DMGDH), choline-phosphate cytidylyltransferase A (PCYT1A), and phosphatidylethanolamine N-..
  9. Lawson J, Park S, Mattison A, Yan J, Reed L. Cloning, expression, and properties of the regulatory subunit of bovine pyruvate dehydrogenase phosphatase. J Biol Chem. 1997;272:31625-9 pubmed
    ..A BLAST search of protein data bases revealed that PDPr is distantly related to the mitochondrial flavoprotein dimethylglycine dehydrogenase, which functions in choline degradation.

More Information

Publications19

  1. Pyo J, Nah J, Kim H, Chang J, Song Y, Yang D, et al. Protection of cardiomyocytes from ischemic/hypoxic cell death via Drbp1 and pMe2GlyDH in cardio-specific ARC transgenic mice. J Biol Chem. 2008;283:30707-14 pubmed publisher
    ..Among them, expressional regulation of developmentally regulated RNA-binding protein 1 (Drbp1) or the dimethylglycine dehydrogenase precursor (pMe(2)GlyDH) affected hypoxic death of cardiomyocytes...
  2. Parker A. A single arginine residue is required for the interaction of the electron transferring flavoprotein (ETF) with three of its dehydrogenase partners. Mol Cell Biochem. 2003;254:91-100 pubmed
  3. Toogood H, van Thiel A, Scrutton N, Leys D. Stabilization of non-productive conformations underpins rapid electron transfer to electron-transferring flavoprotein. J Biol Chem. 2005;280:30361-6 pubmed
    ..modulated rates of interprotein electron transfer with both medium chain acyl-CoA dehydrogenase and dimethylglycine dehydrogenase. The crystal structure of free E165betaA ETF is essentially identical to that of wild-type ETF, but ..
  4. Brizio C, Brandsch R, Douka M, Wait R, Barile M. The purified recombinant precursor of rat mitochondrial dimethylglycine dehydrogenase binds FAD via an autocatalytic reaction. Int J Biol Macromol. 2008;42:455-62 pubmed publisher
    The precursor of the rat mitochondrial flavoenzyme dimethylglycine dehydrogenase (Me(2)GlyDH) has been produced in Escherichia coli as a C-terminally 6-His-tagged fusion protein, purified by one-step affinity chromatography and ..
  5. Brizio C, Barile M, Brandsch R. Flavinylation of the precursor of mitochondrial dimethylglycine dehydrogenase by intact and solubilised mitochondria. FEBS Lett. 2002;522:141-6 pubmed
    The flavinylation and the presequence processing of the mitochondrial matrix enzyme dimethylglycine dehydrogenase (Me(2)GlyDH) were investigated with the reticulocyte lysate translated precursor (pMe(2)GlyDH) added to solubilised ..
  6. Hoffmann L, Brauers G, Gehrmann T, Haussinger D, Mayatepek E, Schliess F, et al. Osmotic regulation of hepatic betaine metabolism. Am J Physiol Gastrointest Liver Physiol. 2013;304:G835-46 pubmed publisher
    ..various genes involved in betaine metabolism, in particular of betaine homocysteine methyltransferase, dimethylglycine dehydrogenase, and sarcosine dehydrogenase...
  7. Lang H, Polster M, Brandsch R. Rat liver dimethylglycine dehydrogenase. Flavinylation of the enzyme in hepatocytes in primary culture and characterization of a cDNA clone. Eur J Biochem. 1991;198:793-9 pubmed
    b>Dimethylglycine dehydrogenase (Me2GlyDH), an enzyme of choline catabolism specifically expressed in the mammalian liver, was analyzed in rat hepatocytes in culture...
  8. Binzak B, Vockley J, Jenkins R, Vockley J. Structure and analysis of the human dimethylglycine dehydrogenase gene. Mol Genet Metab. 2000;69:181-7 pubmed
    b>Dimethylglycine dehydrogenase (DMGDH; E.C. 1.5.99.2) is an enzyme involved in the catabolism of choline, catalyzing the oxidative demethylation of dimethylglycine (DMG) to form sarcosine. Subsequently, sarcosine dehydrogenase (SDH; E.C...
  9. Otto A, Stoltz M, Sailer H, Brandsch R. Biogenesis of the covalently flavinylated mitochondrial enzyme dimethylglycine dehydrogenase. J Biol Chem. 1996;271:9823-9 pubmed
    Rat dimethylglycine dehydrogenase (Me2GlyDH) was used as model protein to study the biogenesis of a covalently flavinylated mitochondrial enzyme...
  10. Wittwer A, Wagner C. Identification of folate binding protein of mitochondria as dimethylglycine dehydrogenase. Proc Natl Acad Sci U S A. 1980;77:4484-8 pubmed
    ..This protein also displays dimethylglycine dehydrogenase [N,N-dimethylglycine: (acceptor) oxidoreductase (demethylating), EC 1.5.99...