CATH Classification

Domain Context

CATH Clusters

Superfamily Aminomethyltransferase beta-barrel domains
Functional Family dimethylglycine dehydrogenase, mitochondrial

Enzyme Information

1.5.8.4
Dimethylglycine dehydrogenase.
based on mapping to UniProt Q9UI17
N,N-dimethylglycine + 5,6,7,8-tetrahydrofolate + electron-transfer flavoprotein = sarcosine + 5,10-methylenetetrahydrofolate + reduced electron-transfer flavoprotein.
-!- An imine intermediate is channeled from the FAD binding site to the 5,6,7,8-tetrahydrofolate binding site through a 40 A tunnel. -!- In the absence of 5,6,7,8-tetrahydrofolate the enzyme forms formaldehyde. -!- Formerly EC 1.5.99.2.

UniProtKB Entries (1)

Q9UI17
M2GD_HUMAN
Homo sapiens
Dimethylglycine dehydrogenase, mitochondrial

PDB Structure

PDB 5L46
External Links
Method X-RAY DIFFRACTION
Organism
Primary Citation
Structure and biochemical properties of recombinant human dimethylglycine dehydrogenase and comparison to the disease-related H109R variant.
Augustin, P., Hromic, A., Pavkov-Keller, T., Gruber, K., Macheroux, P.
Febs J.