EC 1.5.8.4 - Dimethylglycine dehydrogenase

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IntEnz Enzyme Nomenclature
EC 1.5.8.4

Names

Accepted name:
dimethylglycine dehydrogenase
Other names:
N,N-dimethylglycine oxidase
N,N-dimethylglycine:(acceptor) oxidoreductase (demethylating)
Me2GlyDH
N,N-dimethylglycine:electron-transfer flavoprotein oxidoreductase (demethylating)
Systematic name:
N,N-dimethylglycine,5,6,7,8-tetrahydrofolate:electron-transferflavoprotein oxidoreductase (demethylating,5,10-methylenetetrahydrofolate-forming)

Reaction

Cofactor

Comments:

A flavoprotein, containing a histidyl(Nπ)-(8α)FAD linkage at position 91 in the human protein. An imine intermediate is channeled from the FAD binding site to the 5,6,7,8-tetrahydrofolate binding site through a 40 Å tunnel [7,8,9]. In the absence of 5,6,7,8-tetrahydrofolate the enzyme forms formaldehyde [7,9].

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Structural data: CSA , EC2PDB
Gene Ontology: GO:0047865
CAS Registry Number: 37256-30-7
UniProtKB/Swiss-Prot:

References

  1. Frisell, W.R. and MacKenzie, C.G.
    Separation and purification of sarcosine dehydrogenase and dimethylglycine dehydrogenase.
    J. Biol. Chem. 237: 94-98 (1962). [PMID: 13895406]
  2. Hoskins, D.D. and MacKenzie, C.G.
    Solubilization and electron transfer flavoprotein requirement of mitochondrial sarcosine dehydrogenase and dimethylglycine dehydrogenase.
    J. Biol. Chem. 236: 177-183 (1961). [PMID: 13716069]
  3. 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. 37: 434-442 (2004). [PMID: 15358367]
  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. 42: 455-462 (2008). [PMID: 18423846]
  5. Wittwer, A. J., Wagner, C.
    Identification of the folate-binding proteins of rat liver mitochondria as dimethylglycine dehydrogenase and sarcosine dehydrogenase. Purification and folate-binding characteristics.
    J. Biol. Chem. 256: 4102-4108 (1981). [PMID: 6163777]
  6. Wittwer, A. J., Wagner, C.
    Identification of the folate-binding proteins of rat liver mitochondria as dimethylglycine dehydrogenase and sarcosine dehydrogenase. Flavoprotein nature and enzymatic properties of the purified proteins.
    J. Biol. Chem. 256: 4109-4115 (1981). [PMID: 6163778]
  7. Porter, D. H., Cook, R. J., Wagner, C.
    Enzymatic properties of dimethylglycine dehydrogenase and sarcosine dehydrogenase from rat liver.
    Arch. Biochem. Biophys. 243: 396-407 (1985). [PMID: 2417560]
  8. Luka, Z., Pakhomova, S., Loukachevitch, L. V., Newcomer, M. E., Wagner, C.
    Folate in demethylation: the crystal structure of the rat dimethylglycine dehydrogenase complexed with tetrahydrofolate.
    Biochem. Biophys. Res. Commun. 449: 392-398 (2014). [PMID: 24858690]
  9. Augustin, P., Hromic, A., Pavkov-Keller, T., Gruber, K., Macheroux, P.
    Structure and biochemical properties of recombinant human dimethylglycine dehydrogenase and comparison to the disease-related H109R variant.
    FEBS J. 283: 3587-3603 (2016). [PMID: 27486859]

[EC 1.5.8.4 created 1972 as EC 1.5.99.2, transferred 2012 to EC 1.5.8.4, modified 2017]