KEGG   ENZYME: 1.5.8.4
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
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)
R01565
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)
CATY: 105599277(DMGDH)
PANU: 101021742(DMGDH)
RRO: 104662973(DMGDH)
RBB: 108528899(DMGDH)
TFN: 117065309(DMGDH)
PTEH: 111544504(DMGDH)
CJC: 100393734(DMGDH)
SBQ: 101045195(DMGDH)
MMUR: 105872621(DMGDH)
MMU: 74129(Dmgdh)
MCAL: 110307602(Dmgdh)
MPAH: 110329016(Dmgdh)
RNO: 245961(Dmgdh)
MCOC: 116083192(Dmgdh)
MUN: 110539648(Dmgdh)
CGE: 100759503(Dmgdh)
PLEU: 114694633(Dmgdh)
NGI: 103750305(Dmgdh)
HGL: 101722315(Dmgdh)
CCAN: 109675254(Dmgdh)
OCU: 100340207(DMGDH)
TUP: 102489576(DMGDH)
CFA: 488935(DMGDH)
VVP: 112919589(DMGDH)
VLG: 121489124(DMGDH)
AML: 100466333(DMGDH)
UMR: 103681493(DMGDH)
UAH: 113254981(DMGDH)
ORO: 101373953(DMGDH)
ELK: 111148380
MPUF: 101682529(DMGDH)
EJU: 114217588(DMGDH)
MLX: 118016932(DMGDH)
FCA: 101089168(DMGDH)
PTG: 102963363(DMGDH)
PPAD: 109274942(DMGDH)
AJU: 106974795(DMGDH)
HHV: 120242009(DMGDH)
BTA: 504453(DMGDH)
BOM: 102287393(DMGDH)
BIU: 109564663(DMGDH)
BBUB: 102393250(DMGDH)
CHX: 102189681(DMGDH)
OAS: 101113594(DMGDH)
ODA: 120852578(DMGDH)
SSC: 100525563(DMGDH)
CFR: 102522919(DMGDH)
CDK: 105084826(DMGDH)
BACU: 103000721(DMGDH)
LVE: 103081304(DMGDH)
OOR: 101281736(DMGDH)
DLE: 111184963(DMGDH)
PCAD: 102991899(DMGDH)
ECB: 100073267(DMGDH)
EPZ: 103548668(DMGDH)
EAI: 106841177(DMGDH)
MYB: 102246690(DMGDH)
MYD: 102758327(DMGDH)
MMYO: 118656692(DMGDH)
MNA: 107535045(DMGDH)
HAI: 109381891(DMGDH)
DRO: 112307504(DMGDH)
SHON: 118977291(DMGDH)
AJM: 119048139(DMGDH)
MMF: 118641668(DMGDH)
PALE: 102895924(DMGDH)
RAY: 107497232(DMGDH)
MJV: 108398417(DMGDH)
TOD: 119260223(DMGDH)
LAV: 100671550(DMGDH)
TMU: 101355257
MDO: 100032620(DMGDH)
SHR: 100931635(DMGDH)
PCW: 110216593(DMGDH)
OAA: 100088110(DMGDH)
GGA: 416370(DMGDH)
PCOC: 116239924(DMGDH)
CJO: 107305932(DMGDH)
NMEL: 110389379(DMGDH)
APLA: 101802594(DMGDH)
ACYG: 106042090(DMGDH)
TGU: 100231246(DMGDH)
LSR: 110470993(DMGDH)
SCAN: 103819544(DMGDH)
PMOA: 120502443(DMGDH)
GFR: 102044591(DMGDH)
FAB: 101806587(DMGDH)
PHI: 102112577(DMGDH)
PMAJ: 107216431(DMGDH)
CCAE: 111941402(DMGDH)
CCW: 104695710(DMGDH)
ETL: 114056907(DMGDH)
FPG: 101910969(DMGDH)
FCH: 102049188(DMGDH)
CLV: 102091750(DMGDH)
EGZ: 104124898(DMGDH)
NNI: 104017622(DMGDH)
ACUN: 113489761(DMGDH)
PADL: 103919764(DMGDH)
ASN: 102383441(DMGDH)
AMJ: 102561678(DMGDH)
CPOO: 109307687(DMGDH)
GGN: 109297739(DMGDH)
PSS: 102445412(DMGDH)
CMY: 102947394(DMGDH)
CPIC: 101938519(DMGDH)
TST: 117878733(DMGDH)
CABI: 116821376(DMGDH) 116823757
ACS: 100564503(dmgdh)
PVT: 110073960(DMGDH)
PBI: 103049444(DMGDH)
PMUR: 107287877(DMGDH)
TSR: 106544490(DMGDH)
PGUT: 117679271(DMGDH)
PMUA: 114606167(DMGDH)
ZVI: 118076697(DMGDH)
GJA: 107117472(DMGDH)
XLA: 446949(dmgdh.L)
XTR: 100492188(dmgdh)
NPR: 108799972(DMGDH)
DRE: 100141496(dmgdh)
SANH: 107694541(dmgdh)
SGH: 107574100(dmgdh)
IPU: 108279027(dmgdh)
PHYP: 113535384(dmgdh)
AMEX: 103034486(dmgdh)
EEE: 113573718(dmgdh)
TRU: 101062880(dmgdh)
LCO: 104936285(dmgdh)
NCC: 104966861(dmgdh)
ELY: 117269427(dmgdh)
PLEP: 121960316(dmgdh)
SLUC: 116062809(dmgdh)
ECRA: 117959334(dmgdh)
PFLV: 114570945(dmgdh)
GAT: 120832186(dmgdh)
MSAM: 119914232(dmgdh)
CUD: 121525709(dmgdh)
MZE: 101476130(dmgdh)
ONL: 100707764(dmgdh)
OAU: 116315576(dmgdh)
OLA: 101169624(dmgdh)
OML: 112162824(dmgdh)
XMA: 102227264(dmgdh)
XCO: 114150099(dmgdh)
XHE: 116724977(dmgdh)
PRET: 103473305(dmgdh)
CVG: 107093770(dmgdh)
CTUL: 119788279(dmgdh)
NFU: 107374847(dmgdh)
KMR: 108251297(dmgdh)
ALIM: 106515070(dmgdh)
AOCE: 111570345(dmgdh)
CSEM: 103390008(dmgdh)
POV: 109626536(dmgdh)
LCF: 108895442(dmgdh)
SDU: 111239025(dmgdh)
SLAL: 111666799(dmgdh)
XGL: 120806343(dmgdh)
HCQ: 109517687(dmgdh)
BPEC: 110157318(dmgdh)
MALB: 109953004(dmgdh)
SASA: 106568126(dmgdh)
OTW: 112220276(dmgdh)
OMY: 110523447(dmgdh)
SALP: 111961762
ELS: 105015173(dmgdh)
SFM: 108925069(dmgdh)
PKI: 111849812(dmgdh)
LOC: 102682151(dmgdh)
PSPA: 121305151(dmgdh)
LCM: 102353346
CMK: 103177764(dmgdh)
SPU: 587447
PVM: 113817447
HAME: 121878893
EAF: 111708869
DSV: 119442633
RMP: 119188313
PTEP: 107451370
SDM: 118188531
CEL: CELE_Y37E3.17(Y37E3.17)
CBR: CBG22093
BMY: BM_BM6398(Bm6398)
LAK: 106166448
NVE: 5512014
EPA: 110231395
ATEN: 116305412
ADF: 107342978
AMIL: 114953801
DGT: 114533878
HMG: 100209590
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
DALK: DSCA_57620
AMIH: CO731_02954(gcvT_6) CO731_03008(gcvT_9) CO731_03534(gcvT_11)
SFD: USDA257_c45740(gcvT5)
ATU: Atu1632
AVI: Avi_2727
RHT: NT26_1995
MMED: Mame_04001(gcvT_3)
PSF: PSE_p0029
LABT: FIU93_10825(mlr1)
RUT: FIU92_00690(mlr1) FIU92_03040(mlr3) FIU92_05050(mlr4) FIU92_06380(mlr5) FIU92_10495(mlr6) FIU92_12160(mlr7) FIU92_14470(mlr9) FIU92_18060(mlr10) FIU92_20150(mlr14)
RLI: RLO149_c023240(dmgdh1) RLO149_c027920(dmgdh2) RLO149_c034400(dmgdh3) RLO149_c039110(dmgdh4) RLO149_p940550(dmgdh)
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)
RID: RIdsm_01012(gcvT_2) RIdsm_01919(gcvT_6) RIdsm_02570(gcvT_9) RIdsm_02572(gcvT_10) RIdsm_02862(gcvT_11) RIdsm_04377(gcvT_13) RIdsm_04955(gcvT_17) RIdsm_05137(gcvT_18) RIdsm_05412(gcvT_19)
ROH: FIU89_01700(mlr1) FIU89_04725(mlr3) FIU89_05485(mlr5) FIU89_09085(mlr6) FIU89_09090(mlr7)
MARU: FIU81_03420(mlr)
ROT: FIV09_01580(mlr1)
RSP: RSP_3566
PDE: Pden_1940
RSU: NHU_00780
PALW: PSAL_009130(abo_1) PSAL_018780(abo_2)
SWI: Swit_0939
SPHD: HY78_21825
SMIC: SmB9_18450
ANH: A6F65_00140(mlr_2)
 » show all
Reference
1  [PMID:13895406]
  Authors
FRISELL WR, MACKENZIE CG.
  Title
Separation and purification of sarcosine dehydrogenase and dimethylglycine dehydrogenase.
  Journal
J Biol Chem 237:94-8 (1962)
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 236:177-83 (1961)
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 256:4102-8 (1981)
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 256:4109-15 (1981)
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 243:396-407 (1985)
DOI:10.1016/0003-9861(85)90516-8
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 37:434-42 (2004)
DOI:10.1016/j.pep.2004.06.011
  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 42:455-62 (2008)
DOI:10.1016/j.ijbiomac.2008.03.001
  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 449:392-8 (2014)
DOI:10.1016/j.bbrc.2014.05.064
  Sequence
[rno:245961]
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 283:3587-3603 (2016)
DOI:10.1111/febs.13828
  Sequence
[hsa:29958]
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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