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
ec00260  Glycine, serine and threonine metabolism
ec01100  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)
ORO: 101373953(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)
OOR: 101281736(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)
TMU: 101355257
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)
EGZ: 104124898(DMGDH)
AAM: 106489320
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)
SANH: 107694541(dmgdh)
SGH: 107574100(dmgdh)
CCAR: 109046308(dmgdh)
IPU: 108279027(dmgdh)
AMEX: 103034486(dmgdh)
TRU: 101062880(dmgdh)
LCO: 104936285(dmgdh)
NCC: 104966861(dmgdh)
MZE: 101476130(dmgdh)
OLA: 101169624(dmgdh)
XMA: 102227264(dmgdh)
PRET: 103473305(dmgdh)
NFU: 107374847(dmgdh)
CSEM: 103390008(dmgdh)
LCF: 108895442(dmgdh)
HCQ: 109517687(dmgdh)
BPEC: 110157318(dmgdh)
SASA: 106568126(dmgdh)
ELS: 105015173(dmgdh)
SFM: 108925069(dmgdh)
LCM: 102353346
CMK: 103177764(dmgdh)
SPU: 587447
CEL: CELE_Y37E3.17(Y37E3.17)
CBR: CBG22093
BMY: Bm1_17220
LAK: 106166448
EPA: 110231395
ADF: 107342978
HMG: 100209590
AQU: 100637773
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: SPRG_07877
GAI: IMCC3135_12455(abo_3) IMCC3135_18055(abo_4) IMCC3135_27130(abo_5)
BGO: BM43_4044
SFD: USDA257_c45740(gcvT5)
ATU: Atu1632
AVI: Avi_2727
RHT: NT26_1995
MMED: Mame_04001(gcvT_3)
RSP: RSP_3566
RLI: RLO149_c023240(dmgdh1) RLO149_c027920(dmgdh2) RLO149_c034400(dmgdh3) RLO149_c039110(dmgdh4) RLO149_p940550(dmgdh)
PDE: Pden_1940
PSF: PSE_p0029
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)
PHP: PhaeoP97_00680(dmgdH3) PhaeoP97_01401(dmgdH1) PhaeoP97_03223(dmgdH4) PhaeoP97_03709(dmgdH)
PPIC: PhaeoP14_01307(dmgdH1) PhaeoP14_02584(dmgdH3) PhaeoP14_03111(dmgdH4) PhaeoP14_03603(dmgdH)
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)
RSU: NHU_00780
SWI: Swit_0939
SPHD: HY78_21825
ANH: A6F65_00140(mlr_2)
 » 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: 1.5.8.4
IUBMB Enzyme Nomenclature: 1.5.8.4
ExPASy - ENZYME nomenclature database: 1.5.8.4
BRENDA, the Enzyme Database: 1.5.8.4
CAS: 37256-30-7

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