Entry
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
Orthology
K00315 dimethylglycine dehydrogenase
Genes
CABI : 116821376(DMGDH) 116823757
CAUA : 113080753 113090445
MASI : 127428970 127430822
TROS : 130550719 130550721
CCLU : 121541556 121582648(dmgdh)
ARUT : 117403612 117404061
BFO : 118409370 118409371 118409372
BBEL : 109475888 109476278
APLC : 110987881 110988117 110988587
PPOI : 119096221 119096222
RSAN : 119387354 119387359
CEL : CELE_Y37E3.17(Y37E3.17)
PDAM : 113682317 113683486
SPIS : 111324311 111327844
GAI : IMCC3135_12455(abo_3) IMCC3135_18055(abo_4) IMCC3135_27130(abo_5)
MRZ : KDW95_13055 KDW95_18085
TSN : W908_00470 W908_00955 W908_05595 W908_07550
THIN : CRN91_00510 CRN91_00900 CRN91_02465 CRN91_02630
MLO : mll1517 mll1653 mll2449 mlr1213 mlr1280
MLN : A9174_01365 A9174_12360 A9174_18060 A9174_18565 A9174_20015 A9174_20275 A9174_30160
MCI : Mesci_0276 Mesci_3080 Mesci_3179 Mesci_3332 Mesci_3392 Mesci_3401 Mesci_5747
MCIC : A4R28_09725 A4R28_09770 A4R28_10085 A4R28_10870 A4R28_12265 A4R28_26990
MOP : Mesop_3464 Mesop_3570 Mesop_3782 Mesop_3823 Mesop_3876 Mesop_6323
MAM : Mesau_03350 Mesau_03612 Mesau_03663 Mesau_05797
MAMO : A6B35_09220 A6B35_10505 A6B35_12565 A6B35_21685 A6B35_21955 A6B35_23330
MESW : A9K65_001380 A9K65_016715 A9K65_017220 A9K65_018025 A9K65_018325 A9K65_018375
MESM : EJ066_25580 EJ066_25820 EJ066_27085
MESP : C1M53_21985 C1M53_24225 C1M53_24480
MHUA : MCHK_0600 MCHK_2856 MCHK_2910 MCHK_3149 MCHK_3256 MCHK_4075 MCHK_6281
MJR : EB229_01390 EB229_12225 EB229_18325 EB229_18850 EB229_19775 EB229_20045 EB229_30760
MERD : EB233_01470 EB233_12470 EB233_15650 EB233_16180 EB233_17110 EB233_17420 EB233_26985
MESR : FGU64_05200 FGU64_05430
MOH : IHQ72_13450 IHQ72_16530 IHQ72_17795 IHQ72_18020
MMEI : LRP31_16770 LRP31_18015 LRP31_18255 LRP31_33515
NKI : KW403_01365 KW403_16460
NRH : T8J41_13240 T8J41_16880
NTU : NTH_00451(abo_1) NTH_00487(abo_2)
MESO : BSQ44_15470 BSQ44_15795
AAK : AA2016_2973 AA2016_3040
AMIH : CO731_02954(gcvT_6) CO731_03008(gcvT_9) CO731_03534(gcvT_11)
AMIS : Amn_33920 Amn_34580
ANJ : AMD1_2874(gcvT) AMD1_2933(gcvT) AMD1_3493(gcvT)
PHT : BLM14_05370 BLM14_05485 BLM14_08065
PHYL : HB779_17815 HB779_17945
KMN : HW532_09130 HW532_10840
SMK : Sinme_2255 Sinme_6001
SMQ : SinmeB_2099 SinmeB_6321
SMX : SM11_chr0983 SM11_pC0842
SMI : BN406_02099 BN406_04544
SMEG : C770_GR4Chr2334 C770_GR4pC0569
SMEL : SM2011_a1483 SM2011_c01663
SMER : DU99_12730 DU99_21970
RHI : NGR_b16530 NGR_c21820
SFD : USDA257_c45740(gcvT5)
SIX : BSY16_102 BSY16_4802 BSY16_522
SKM : PZL22_002610 PZL22_005008
EAD : OV14_3709 OV14_4043 OV14_a0111
EAH : FA04_11570 FA04_13205
ECAA : J3R84_11705 J3R84_13580 J3R84_27260
ENP : JVX98_08120 JVX98_26555
RJG : CCGE525_12245 CCGE525_15975
RRHO : PR018_10905 PR018_18620
RTU : PR017_11065 PR017_27300
NEN : NCHU2750_25890 NCHU2750_44930
SOJ : K6301_09700 K6301_11200 K6301_20490
SSUM : Q9314_15460 Q9314_16950 Q9314_19625
HOE : IMCC20628_00539 IMCC20628_01684
PSCQ : KHQ08_09115 KHQ08_11380
LNE : FZC33_05130 FZC33_07605 FZC33_17220 FZC33_19170
NOH : G5V57_01050 G5V57_25045 G5V57_32635
LABR : CHH27_14530 CHH27_15475 CHH27_24580
LABP : FJ695_07950 FJ695_09645 FJ695_25365
RPOP : K1718_05600 K1718_06470 K1718_13125 K1718_21220
LIZ : LGH83_00890 LGH83_07170
SIL : SPO0207 SPO0544 SPO0564 SPO0635 SPO1592 SPO1628 SPO3400 SPOA0063 SPOA0310
SIT : TM1040_0486 TM1040_1426 TM1040_2063
RUA : D1823_00950 D1823_02745 D1823_06845 D1823_08205 D1823_10250 D1823_12790 D1823_16990 D1823_20780
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)
RCON : K3740_07250 K3740_12625 K3740_13670 K3740_14665 K3740_15760 K3740_16870 K3740_22425 K3740_22620
RUY : NOR97_00375 NOR97_02940 NOR97_05020 NOR97_05805 NOR97_06355 NOR97_10080 NOR97_11475 NOR97_13420 NOR97_17330
JAN : Jann_0919 Jann_1481 Jann_1596 Jann_2488 Jann_2805
RDE : RD1_0019 RD1_0607 RD1_1345 RD1_3093 RD1_3434
RLI : RLO149_c023240(dmgdh1) RLO149_c027920(dmgdh2) RLO149_c034400(dmgdh3) RLO149_c039110(dmgdh4) RLO149_p940550(dmgdh)
RPON : G3256_01370 G3256_09520 G3256_09530 G3256_10965 G3256_14570 G3256_16305
RFU : ROLI_003500(abo_1) ROLI_003540(dmg) ROLI_022160(abo_3) ROLI_023610(abo_4) ROLI_031200(abo_6)
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 : Gal_00601 Gal_01999 Gal_03372 Gal_03876
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)
PHQ : D1820_03020 D1820_08825 D1820_11350 D1820_17765
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)
OCT : FTO60_03970 FTO60_07420 FTO60_07425 FTO60_12415 FTO60_13430 FTO60_15110
LMD : METH_03735 METH_03940 METH_10325 METH_15465 METH_18800 METH_19120 METH_21940 METH_22240
LEJ : ETW24_01830 ETW24_04590 ETW24_05260 ETW24_11035 ETW24_15600 ETW24_18640 ETW24_20765 ETW24_21005
LAQU : R2C4_02365 R2C4_03560 R2C4_07700 R2C4_13145 R2C4_19080
LEV : ETW23_01350 ETW23_14330 ETW23_14920 ETW23_15480 ETW23_21115 ETW23_22940
LCAE : K3721_03875 K3721_03965 K3721_09475 K3721_13810 K3721_15285 K3721_16875 K3721_19865 K3721_20370
RED : roselon_00386 roselon_02666 roselon_03053
MALG : MALG_00176 MALG_02067 MALG_02872 MALG_05031
CON : TQ29_02695 TQ29_08680
SUAM : BOO69_09345 BOO69_09355 BOO69_14040 BOO69_14970 BOO69_16555
SULZ : C1J03_02070 C1J03_02180 C1J03_12000 C1J03_12980 C1J03_12985 C1J03_18530 C1J03_24870
SULI : C1J05_00620 C1J05_01555 C1J05_03595 C1J05_03615 C1J05_06305 C1J05_09340 C1J05_09350 C1J05_17060 C1J05_17365
SULD : B5M07_03755 B5M07_09395 B5M07_10375
SMED : JNX03_03690 JNX03_03695 JNX03_10375 JNX03_12625 JNX03_14895 JNX03_17695
SINL : DSM14862_01486(abo_1) DSM14862_01603(abo_3) DSM14862_02797(abo_4)
SFAV : PL335_07650 PL335_13005
SUAL : KDD17_01820 KDD17_04860 KDD17_16700 KDD17_17090
SPSE : SULPSESMR1_00305(abo) SULPSESMR1_01729(abo) SULPSESMR1_01730(abo) SULPSESMR1_02942(abo)
TOM : BWR18_06035 BWR18_06045 BWR18_12820 BWR18_14145 BWR18_15290 BWR18_17915
RMM : ROSMUCSMR3_00197(abo) ROSMUCSMR3_00199(abo) ROSMUCSMR3_01611(dmg) ROSMUCSMR3_02526(abo) ROSMUCSMR3_03060(abo)
ROK : RAK1035_0653 RAK1035_1554 RAK1035_1955 RAK1035_3049 RAK1035_3051
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)
ROM : EI983_07495 EI983_07510 EI983_08870 EI983_10870 EI983_11245 EI983_15160 EI983_16220 EI983_18010
ROH : FIU89_01700(mlr1) FIU89_04725(mlr3) FIU89_05485(mlr5) FIU89_09085(mlr6) FIU89_09090(mlr7)
RPEL : N7U68_01680 N7U68_01695 N7U68_03735 N7U68_04390 N7U68_11165 N7U68_15355 N7U68_15850
AHT : ANTHELSMS3_00322 ANTHELSMS3_02047 ANTHELSMS3_02167 ANTHELSMS3_03636 ANTHELSMS3_03637 ANTHELSMS3_04119(gcvT)
SAGU : CDO87_04200 CDO87_04815 CDO87_18660
THAA : CFI11_10575 CFI11_10585 CFI11_17605 CFI11_17875 CFI11_21505
OCD : FHY55_05645 FHY55_10830
MALU : KU6B_16150 KU6B_29170 KU6B_29180 KU6B_41430 KU6B_42750 KU6B_44080
LALG : LentiSH36_00144(dmgdH3) LentiSH36_00395(dmgdH1_1) LentiSH36_01371(dmgdH) LentiSH36_01372(dmgdH1_2) LentiSH36_02504(dmgdH2) LentiSH36_02924(dmgdH4)
TRY : QF118_09455 QF118_10250 QF118_12630 QF118_12635 QF118_18490
RMAI : MACH21_09120 MACH21_13320 MACH21_24120
RBL : B6K69_00135 B6K69_00670 B6K69_07415
FYT : QF092_02100 QF092_02620 QF092_10265 QF092_15240
PZH : CX676_08395 CX676_11705
PAAK : FIU66_08115 FIU66_16345
PSEW : JHW44_16570 JHW44_16690
PSAP : JHX88_09195 JHX88_14910
PTP : RCA23_c00660 RCA23_c01560 RCA23_c15530 RCA23_c15540 RCA23_c20240 RCA23_c21230
RMB : K529_002195 K529_003550 K529_006445
TSCO : R1T40_01480 R1T40_01935 R1T40_04815 R1T40_15290
DAA : AKL17_0631 AKL17_1896
LVS : LOKVESSMR4R_00303(abo) LOKVESSMR4R_02748(abo)
GEH : HYN69_00790 HYN69_09150
GFU : KM031_00015 KM031_00510 KM031_13160
TAW : EI545_00905 EI545_01500 EI545_13050
SEDI : EBB79_09265 EBB79_11090 EBB79_17395 EBB79_23745
PPSO : QPJ95_05440 QPJ95_19025 QPJ95_22325
BOO : E2K80_03410 E2K80_06725 E2K80_08300 E2K80_09280 E2K80_14235
PPRU : FDP22_07225 FDP22_17495
PAED : G5B38_00855 G5B38_01410 G5B38_03515 G5B38_03530 G5B38_08335 G5B38_08835 G5B38_12785
PAMO : BAR1_13035 BAR1_14675
POZ : I0K15_02065 I0K15_05420
PALW : PSAL_009130(abo_1) PSAL_018780(abo_2)
PSHQ : F3W81_04160 F3W81_20390
PPAF : I8N54_09580 I8N54_15200
CACT : HZ995_04745 HZ995_05725 HZ995_06915 HZ995_07390 HZ995_10220 HZ995_10555 HZ995_15380
THAS : C6Y53_03320 C6Y53_08730 C6Y53_16540
HDH : G5B40_00965 G5B40_01990 G5B40_17880
GCE : KYE46_08385 KYE46_11150 KYE46_13420 KYE46_15840 KYE46_17050
GYJ : AADW23_07395 AADW23_10885 AADW23_14115 AADW23_16135
NSM : JO391_07230 JO391_08970 JO391_15875
ACRO : K3J57_02380 K3J57_13150
AALK : LGT41_0002835 LGT41_0002980 LGT41_0006600 LGT41_0011770
AMYL : QBD29_02625 QBD29_05035 QBD29_06815 QBD29_07555 QBD29_09020 QBD29_15455 QBD29_15880
AMAQ : GO499_13745 GO499_19135
RHOC : QTA57_01540 QTA57_12440 QTA57_14800 QTA57_17780
RBG : BG454_17160 BG454_17495 BG454_17960
BOSG : GKR98_02410 GKR98_05030 GKR98_07065 GKR98_09535 GKR98_13470 GKR98_17125
FAQ : G5B39_05390 G5B39_14330 G5B39_16070
RBX : I3V23_02345 I3V23_07030
SLAA : EUU25_02130 EUU25_02325
HTQ : FRZ44_31630 FRZ44_34370 FRZ44_36700
HADH : FRZ61_16050 FRZ61_33550
NACI : NUH88_07300 NUH88_15070
THAR : T8K17_10120 T8K17_13700
APM : HIMB5_00008670 HIMB5_00008920
APB : SAR116_0544 SAR116_1059 SAR116_1184 SAR116_2173
SNEA : NBZ79_06220 NBZ79_06330 NBZ79_08355 NBZ79_08560
» show all
Taxonomy
Reference
Authors
FRISELL WR, MACKENZIE CG.
Title
Separation and purification of sarcosine dehydrogenase and dimethylglycine dehydrogenase.
Journal
J Biol Chem 237:94-8 (1962)
Reference
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
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
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
Authors
Porter DH, Cook RJ, Wagner C
Title
Enzymatic properties of dimethylglycine dehydrogenase and sarcosine dehydrogenase from rat liver.
Journal
Reference
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
Sequence
Reference
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
Sequence
Reference
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
Sequence
Reference
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
Sequence
Other DBs
ExplorEnz - The Enzyme Database: 1.5.8.4
ExPASy - ENZYME nomenclature database: 1.5.8.4
BRENDA, the Enzyme Database: 1.5.8.4
LinkDB
All DBs