KEGG   ENZYME: 1.5.8.4Help
Entry
EC 1.5.8.4                  Enzyme                                 

Name
dimethylglycine dehydrogenase;
N,N-dimethylglycine oxidase;
N,N-dimethylglycine:(acceptor) oxidoreductase (demethylating);
Me2GlyDH;
N,N-dimethylglycine:electron-transfer flavoprotein oxidoreductase (demethylating)
Class
Oxidoreductases;
Acting on the CH-NH group of donors;
With a flavin or flavoprotein as acceptor
BRITE hierarchy
Sysname
N,N-dimethylglycine,5,6,7,8-tetrahydrofolate:electron-transferflavoprotein oxidoreductase (demethylating,5,10-methylenetetrahydrofolate-forming)
Reaction(IUBMB)
N,N-dimethylglycine + 5,6,7,8-tetrahydrofolate + electron-transfer flavoprotein = sarcosine + 5,10-methylenetetrahydrofolate + reduced electron-transfer flavoprotein [RN:R01565]
Reaction(KEGG)
Substrate
N,N-dimethylglycine [CPD:C01026];
5,6,7,8-tetrahydrofolate [CPD:C00101];
electron-transfer flavoprotein [CPD:C04253]
Product
sarcosine [CPD:C00213];
5,10-methylenetetrahydrofolate [CPD:C00143];
reduced electron-transfer flavoprotein [CPD:C04570]
Comment
A flavoprotein, containing a histidyl(Npi)-(8alpha)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 A tunnel [5,8,9]. In the absence of 5,6,7,8-tetrahydrofolate the enzyme forms formaldehyde [5,9].
History
EC 1.5.8.4 created 1972 as EC 1.5.99.2, transferred 2012 to EC 1.5.8.4, modified 2017
Pathway
Glycine, serine and threonine metabolism
Metabolic pathways
Orthology
K00315  
dimethylglycine dehydrogenase
Genes
HSA: 
29958(DMGDH)
PTR: 
471505(DMGDH)
PPS: 
100985018(DMGDH)
GGO: 
101138464(DMGDH)
PON: 
100457567(DMGDH)
NLE: 
100587925(DMGDH)
MCC: 
709237(DMGDH)
MCF: 
102117996(DMGDH)
CSAB: 
103223602(DMGDH)
RRO: 
104662973(DMGDH)
RBB: 
108528899(DMGDH)
CJC: 
100393734(DMGDH)
SBQ: 
101045195(DMGDH)
MMU: 
74129(Dmgdh)
RNO: 
245961(Dmgdh)
CGE: 
100759503(Dmgdh)
NGI: 
103750305(Dmgdh)
HGL: 
101722315(Dmgdh)
CCAN: 
109675254(Dmgdh)
OCU: 
100340207(DMGDH)
TUP: 
102489576(DMGDH)
CFA: 
488935(DMGDH)
AML: 
100466333(DMGDH)
UMR: 
103681493(DMGDH)
FCA: 
101089168(DMGDH)
PTG: 
102963363(DMGDH)
AJU: 
106974795(DMGDH)
BTA: 
504453(DMGDH)
BOM: 
102287393(DMGDH)
BIU: 
109564663(DMGDH)
PHD: 
102327524(DMGDH)
CHX: 
102189681(DMGDH)
OAS: 
101113594(DMGDH)
SSC: 
100525563(DMGDH)
CFR: 
102522919(DMGDH)
CDK: 
105084826(DMGDH)
BACU: 
103000721(DMGDH)
LVE: 
103081304(DMGDH)
ECB: 
100073267(DMGDH)
EPZ: 
103548668(DMGDH)
EAI: 
106841177(DMGDH)
MYB: 
102246690(DMGDH)
MYD: 
102758327(DMGDH)
HAI: 
109381891(DMGDH)
RSS: 
109445644(DMGDH)
PALE: 
102895924(DMGDH)
LAV: 
100671550(DMGDH)
MDO: 
100032620(DMGDH)
SHR: 
100931635(DMGDH)
OAA: 
100088110(DMGDH)
GGA: 
416370(DMGDH)
CJO: 
107305932(DMGDH)
APLA: 
101802594(DMGDH)
ACYG: 
106042090(DMGDH)
TGU: 
100231246(DMGDH)
GFR: 
102044591(DMGDH)
FAB: 
101806587(DMGDH)
PHI: 
102112577(DMGDH)
PMAJ: 
107216431(DMGDH)
CCW: 
104695710(DMGDH)
FPG: 
101910969(DMGDH)
FCH: 
102049188(DMGDH)
CLV: 
102091750(DMGDH)
AAM: 
ASN: 
102383441(DMGDH)
AMJ: 
102561678(DMGDH)
PSS: 
102445412(DMGDH)
CMY: 
102947394(DMGDH)
CPIC: 
101938519(DMGDH)
ACS: 
100564503(dmgdh)
PVT: 
110073960(DMGDH)
PBI: 
103049444(DMGDH)
GJA: 
107117472(DMGDH)
XLA: 
446949(dmgdh.L)
XTR: 
100492188(dmgdh)
NPR: 
108799972(DMGDH)
DRE: 
100141496(dmgdh)
SRX: 
SANH: 
107694541(dmgdh)
SGH: 
107574100(dmgdh)
IPU: 
108279027(dmgdh)
TRU: 
101062880(dmgdh)
LCO: 
104936285(dmgdh)
NCC: 
104966861(dmgdh)
MZE: 
101476130(dmgdh)
OLA: 
XMA: 
102227264(dmgdh)
CSEM: 
103390008(dmgdh)
LCF: 
108895442(dmgdh)
HCQ: 
109517687(dmgdh)
BPEC: 
110157318(dmgdh)
SASA: 
106568126(dmgdh)
ELS: 
105015173(dmgdh)
SFM: 
108925069(dmgdh)
LCM: 
CMK: 
103177764(dmgdh)
BFO: 
SPU: 
SKO: 
ISC: 
CEL: 
CELE_Y37E3.17(Y37E3.17)
CBR: 
NAI: 
BMY: 
LOA: 
LAK: 
NVE: 
ADF: 
HMG: 
TAD: 
AQU: 
SMIN: 
v1.2.028204.t1(symbB.v1.2.028204.t1) v1.2.028204.t2(symbB.v1.2.028204.t2) v1.2.028204.t3(symbB.v1.2.028204.t3)
SPAR: 
EHX: 
GAI: 
TSN: 
BGO: 
AZA: 
MLO: 
MLN: 
MCI: 
MOP: 
MAM: 
MAMO: 
MESO: 
MESW: 
HOE: 
AAK: 
SME: 
SMK: 
SMQ: 
SMX: 
SMI: 
SMEG: 
SMEL: 
SMER: 
SMD: 
RHI: 
SFH: 
SFD: 
SIX: 
SAME: 
EAD: 
EAH: 
ATU: 
ARA: 
ATF: 
ATA: 
AVI: 
AGR: 
AGC: 
ARO: 
RLT: 
RTR: 
RIR: 
RHL: 
RHT: 
NGL: 
NGG: 
SHZ: 
OCH: 
MEY: 
MAAD: 
MMED: 
Mame_04001(gcvT_3)
RBM: 
SIL: 
SIT: 
RMB: 
RSP: 
RSH: 
RSQ: 
RSK: 
RCP: 
RHP: 
JAN: 
RDE: 
RLI: 
PDE: 
PAMI: 
PYE: 
DSH: 
PSF: 
PGA: 
PGA1_262p01400(dmgdH) PGA1_c14040(dmgdH1) PGA1_c25540(dmgdH2) PGA1_c28230(dmgdH3) PGA1_c33130(dmgdH4)
PGL: 
PGA2_239p1580(dmgdH) PGA2_c13940(dmgdH1) PGA2_c26230(dmgdH2) PGA2_c31400(dmgdH3)
PGD: 
PHP: 
OAT: 
OAN307_c07010(dmgdh5) OAN307_c24050(dmgdh3) OAN307_c24060(dmgdh1) OAN307_c42890(dmgdh2) OAN307_c46320(dmgdh4)
OAR: 
OA238_c04890(dmgdh2) OA238_c19930(dmgdh1) OA238_c19940(dmgdh3) OA238_c34950(dmgdh5) OA238_c40910(dmgdh4)
OTM: 
OSB_05340(mlr_1) OSB_15100(mlr_2) OSB_15120(mlr_3) OSB_26970(mlr_5) OSB_30270(mlr_7)
LMD: 
RED: 
PTP: 
MALG: 
CON: 
RSU: 
RHM: 
HAT: 
LAP: 
LAGG: 
LABR: 
DAA: 
YAN: 
SUAM: 
SPSE: 
DON: 
TOM: 
THW: 
RMM: 
LVS: 
AHT: 
SWI: 
ANH: 
PGV: 
PUB: 
APM: 
APB: 
 » show all
Taxonomy
Reference
1  [PMID:13895406]
  Authors
FRISELL WR, MACKENZIE CG.
  Title
Separation and purification of sarcosine dehydrogenase and dimethylglycine dehydrogenase.
  Journal
J. Biol. Chem. 237 (1962) 94-8.
Reference
2  [PMID:13716069]
  Authors
HOSKINS DD, MACKENZIE CG.
  Title
Solubilization and electron transfer flavoprtein requirement of mitochondrial sarcosine dehydrogenase and dimethylglycine dehydrogenase.
  Journal
J. Biol. Chem. 236 (1961) 177-83.
Reference
3  [PMID:6163777]
  Authors
Wittwer AJ, Wagner C
  Title
Identification of the folate-binding proteins of rat liver mitochondria as dimethylglycine dehydrogenase and sarcosine dehydrogenase. Purification and folate-binding characteristics.
  Journal
J. Biol. Chem. 256 (1981) 4102-8.
Reference
4  [PMID:6163778]
  Authors
Wittwer AJ, Wagner C
  Title
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.
  Journal
J. Biol. Chem. 256 (1981) 4109-15.
Reference
5  [PMID:2417560]
  Authors
Porter DH, Cook RJ, Wagner C
  Title
Enzymatic properties of dimethylglycine dehydrogenase and sarcosine dehydrogenase from rat liver.
  Journal
Arch. Biochem. Biophys. 243 (1985) 396-407.
Reference
6  [PMID:15358367]
  Authors
Brizio C, Brandsch R, Bufano D, Pochini L, Indiveri C, Barile M
  Title
Over-expression in Escherichia coli, functional characterization and refolding of rat dimethylglycine dehydrogenase.
  Journal
Protein. Expr. Purif. 37 (2004) 434-42.
  Sequence
[rno:245961]
Reference
7  [PMID:18423846]
  Authors
Brizio C, Brandsch R, Douka M, Wait R, Barile M
  Title
The purified recombinant precursor of rat mitochondrial dimethylglycine dehydrogenase binds FAD via an autocatalytic reaction.
  Journal
Int. J. Biol. Macromol. 42 (2008) 455-62.
  Sequence
[rno:245961]
Reference
8  [PMID:24858690]
  Authors
Luka Z, Pakhomova S, Loukachevitch LV, Newcomer ME, Wagner C
  Title
Folate in demethylation: the crystal structure of the rat dimethylglycine dehydrogenase complexed with tetrahydrofolate.
  Journal
Biochem. Biophys. Res. Commun. 449 (2014) 392-8.
Reference
9  [PMID:27486859]
  Authors
Augustin P, Hromic A, Pavkov-Keller T, Gruber K, Macheroux P
  Title
Structure and biochemical properties of recombinant human dimethylglycine dehydrogenase and comparison to the disease-related H109R variant.
  Journal
FEBS. J. 283 (2016) 3587-3603.
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
37256-30-7

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