KEGG   ORTHOLOGY: K00288Help
Entry
K00288                      KO                                     

Name
MTHFD
Definition
methylenetetrahydrofolate dehydrogenase (NADP+) / methenyltetrahydrofolate cyclohydrolase / formyltetrahydrofolate synthetase [EC:1.5.1.5 3.5.4.9 6.3.4.3]
Pathway
One carbon pool by folate
Module
M00141  
C1-unit interconversion, eukaryotes
Disease
H00262  
Spina bifida
Brite
KEGG Orthology (KO) [BR:ko00001]
 Metabolism
  Metabolism of cofactors and vitamins
   00670 One carbon pool by folate
    K00288  MTHFD; methylenetetrahydrofolate dehydrogenase (NADP+) / methenyltetrahydrofolate cyclohydrolase / formyltetrahydrofolate synthetase
KEGG modules [BR:ko00002]
 Pathway module
  Nucleotide and amino acid metabolism
   Cofactor and vitamin biosynthesis
    M00141  C1-unit interconversion, eukaryotes
     K00288  MTHFD; methylenetetrahydrofolate dehydrogenase (NADP+) / methenyltetrahydrofolate cyclohydrolase / formyltetrahydrofolate synthetase
Enzymes [BR:ko01000]
 1. Oxidoreductases
  1.5  Acting on the CH-NH group of donors
   1.5.1  With NAD+ or NADP+ as acceptor
    1.5.1.5  methylenetetrahydrofolate dehydrogenase (NADP+)
     K00288  MTHFD; methylenetetrahydrofolate dehydrogenase (NADP+) / methenyltetrahydrofolate cyclohydrolase / formyltetrahydrofolate synthetase
 3. Hydrolases
  3.5  Acting on carbon-nitrogen bonds, other than peptide bonds
   3.5.4  In cyclic amidines
    3.5.4.9  methenyltetrahydrofolate cyclohydrolase
     K00288  MTHFD; methylenetetrahydrofolate dehydrogenase (NADP+) / methenyltetrahydrofolate cyclohydrolase / formyltetrahydrofolate synthetase
 6. Ligases
  6.3  Forming carbon-nitrogen bonds
   6.3.4  Other carbon-nitrogen ligases
    6.3.4.3  formate---tetrahydrofolate ligase
     K00288  MTHFD; methylenetetrahydrofolate dehydrogenase (NADP+) / methenyltetrahydrofolate cyclohydrolase / formyltetrahydrofolate synthetase
Exosome [BR:ko04147]
 Exosomal proteins
  Exosomal proteins of other body fluids (saliva and urine)
   K00288  MTHFD; methylenetetrahydrofolate dehydrogenase (NADP+) / methenyltetrahydrofolate cyclohydrolase / formyltetrahydrofolate synthetase
BRITE hierarchy
Other DBs
Genes
HSA: 
4522(MTHFD1)
PTR: 
452965(MTHFD1)
PPS: 
100986093(MTHFD1)
GGO: 
101145772(MTHFD1)
PON: 
100173036(MTHFD1)
NLE: 
100592446(MTHFD1)
MCC: 
707059(MTHFD1)
MCF: 
102146226(MTHFD1)
CSAB: 
103229163(MTHFD1)
RRO: 
104678419(MTHFD1)
CJC: 
100385373(MTHFD1)
SBQ: 
101050716(MTHFD1)
MMU: 
108156(Mthfd1)
RNO: 
64300(Mthfd1)
CGE: 
100763862(Mthfd1)
NGI: 
103730291 103749265(Mthfd1)
HGL: 
101707403(Mthfd1)
CCAN: 
109691124(Mthfd1)
OCU: 
100347271(MTHFD1)
TUP: 
102473748(MTHFD1)
CFA: 
480352(MTHFD1)
AML: 
100467466(MTHFD1)
UMR: 
103672303(MTHFD1)
FCA: 
101086816(MTHFD1)
PTG: 
102971656(MTHFD1)
AJU: 
106971326(MTHFD1)
BTA: 
534382(MTHFD1)
BOM: 
102275233(MTHFD1)
BIU: 
109565242(MTHFD1)
PHD: 
102334237(MTHFD1)
CHX: 
102186214(MTHFD1)
OAS: 
101109020(MTHFD1)
SSC: 
414382(MTHFD1)
CFR: 
102504065(MTHFD1)
BACU: 
103017005(MTHFD1)
LVE: 
103090767(MTHFD1)
ECB: 
100062154(MTHFD1)
MYB: 
102241254(MTHFD1)
MYD: 
102757978(MTHFD1)
HAI: 
109382053(MTHFD1)
RSS: 
109448237(MTHFD1)
PALE: 
102878910(MTHFD1)
LAV: 
100661814(MTHFD1)
MDO: 
100027684(MTHFD1)
SHR: 
100917465(MTHFD1)
GGA: 
423508(MTHFD1)
MGP: 
100542376(MTHFD1)
CJO: 
107315290(MTHFD1)
APLA: 
101798457(MTHFD1)
TGU: 
100218066(MTHFD1)
GFR: 
102040140(MTHFD1)
FAB: 
101820743(MTHFD1)
PHI: 
102101641(MTHFD1)
CCW: 
104687153(MTHFD1)
FPG: 
101913340(MTHFD1)
FCH: 
102059574(MTHFD1)
CLV: 
102091689(MTHFD1)
AAM: 
106483010(MTHFD1)
ASN: 
102377792(MTHFD1)
AMJ: 
102576903(MTHFD1)
PSS: 
102452269(MTHFD1)
CMY: 
102943369(MTHFD1)
CPIC: 
101941983(MTHFD1)
ACS: 
100553949(mthfd1)
PBI: 
103050569 103051435 103058437(MTHFD1)
GJA: 
107107643 107119326(MTHFD1)
XLA: 
380266(mthfd1.L)
XTR: 
493369(mthfd1)
DRE: 
260441(mthfd1b)
IPU: 
108257577(mthfd1) 108269612(Mthfd1)
TRU: 
101079275 105418662(mthfd1)
TNG: 
GSTEN00008408G001 GSTEN00032425G001
LCO: 
104924805(mthfd1) 104933183
MZE: 
101477222 101486590(mthfd1)
OLA: 
101159402 101174548
XMA: 
102224500(mthfd1)
CSEM: 
103381395 103390162(mthfd1) 103399305
SASA: 
106581504(mthfd1) 106607641
LCM: 
102347033(MTHFD1)
CMK: 
103189752(mthfd1)
BFO: 
BRAFLDRAFT_120483 BRAFLDRAFT_289526
CIN: 
100185664
SPU: 
589933(MTHFD1)
SKO: 
100366866
DME: 
Dmel_CG4067(pug)
DPO: 
Dpse_GA13173
DAN: 
Dana_GF17545
DER: 
Dere_GG17628
DPE: 
Dper_GL11980
DSE: 
Dsec_GM23891
DSI: 
Dsimw501_GD18702(Dsim_GD18702)
DWI: 
Dwil_GK11804
DYA: 
Dyak_GE26043
DGR: 
Dgri_GH14901
DMO: 
Dmoj_GI23694
DVI: 
Dvir_GJ23253
MDE: 
101891351
AGA: 
AgaP_AGAP002830
AAG: 
AaeL_AAEL006085
CQU: 
CpipJ_CPIJ014099
AME: 
550749(pug)
BIM: 
100741383
BTER: 
100644014
SOC: 
105195662
AEC: 
105144492
PBAR: 
105431753
HST: 
105192491
CFO: 
105252037
NVI: 
100118909
TCA: 
656820 656994
BMOR: 
101743494
PXY: 
105382326 105390540
API: 
100167020
PHU: 
Phum_PHUM025050 Phum_PHUM456190
DPX: 
DAPPUDRAFT_106818 DAPPUDRAFT_198836 DAPPUDRAFT_248714 DAPPUDRAFT_248715 DAPPUDRAFT_326336 DAPPUDRAFT_67147
CEL: 
CELE_K07E3.4(K07E3.4)
NAI: 
NECAME_01976 NECAME_01978 NECAME_02558
TSP: 
Tsp_00651
HRO: 
HELRODRAFT_104956
LGI: 
LOTGIDRAFT_201055
CRG: 
105345937
OBI: 
106867868 106869903
SMM: 
Smp_147650
OVI: 
T265_00055
NVE: 
NEMVE_v1g158170
ADF: 
107335679 107337241
HMG: 
100203891 100209510 101238037
TAD: 
TRIADDRAFT_49681
AQU: 
100634307
EGR: 
104422249
PPER: 
18773159
SIND: 
105180013
CCP: 
CHC_T00009198001
SCE: 
YBR084W(MIS1) YGR204W(ADE3)
AGO: 
AGOS_ACL121C AGOS_AER178W
ERC: 
Ecym_7378 Ecym_7435
KLA: 
KLLA0E12475g KLLA0E16545g
LTH: 
KLTH0B01496g KLTH0E14366g
VPO: 
Kpol_1050p36 Kpol_243p3
ZRO: 
ZYRO0G11550g ZYRO0G12936g
CGR: 
CAGL0K00913g CAGL0M01694g
NCS: 
NCAS_0A07290(NCAS0A07290) NCAS_0I01350(NCAS0I01350)
NDI: 
NDAI_0A07650(NDAI0A07650) NDAI_0D02270(NDAI0D02270)
TPF: 
TPHA_0A03820(TPHA0A03820) TPHA_0A04840(TPHA0A04840)
TBL: 
TBLA_0B07610(TBLA0B07610) TBLA_0I02160(TBLA0I02160)
TDL: 
TDEL_0E04950(TDEL0E04950) TDEL_0F02070(TDEL0F02070)
KAF: 
KAFR_0B04020(KAFR0B04020) KAFR_0C00240(KAFR0C00240)
PPA: 
PAS_chr1-4_0632
DHA: 
DEHA2E18414g DEHA2G23650g
PIC: 
PICST_80013(MIS1) PICST_88119(ADE3)
PGU: 
PGUG_01248 PGUG_04085
SPAA: 
SPAPADRAFT_134523(ADE3) SPAPADRAFT_49629
LEL: 
LELG_00053 LELG_03230
CAL: 
CAALFM_CR00080WA(MIS12) CAALFM_CR07010WA(MIS11)
CTP: 
CTRG_03112
COT: 
CORT_0A00680 CORT_0D04820
CDU: 
CD36_25100 CD36_32540
CTEN: 
CANTEDRAFT_99240 CANTEDRAFT_99366
YLI: 
YALI0E01056g YALI0F30745g
CLU: 
CLUG_02470 CLUG_03993
CAUR: 
QG37_01301 QG37_03990
NCR: 
NCU03877
NTE: 
NEUTE1DRAFT88125(NEUTE1DRAFT_88125)
SMP: 
SMAC_06300
PAN: 
PODANSg944
TTT: 
THITE_2110770
MTM: 
MYCTH_2307368
CTHR: 
CTHT_0045900
MGR: 
MGG_01014
TMN: 
UCRPA7_1000
FGR: 
FGSG_10267
FPU: 
FPSE_03604
FVR: 
FVEG_02948
FOX: 
FOXG_05185
NHE: 
NECHADRAFT_60993
TRE: 
TRIREDRAFT_75890
MAW: 
MAC_07740
MAJ: 
MAA_00020
VAL: 
VDBG_04290
VDA: 
VDAG_01520
CFJ: 
CFIO01_11507
ELA: 
UCREL1_10121
SSL: 
SS1G_03895
BFU: 
BC1G_04451
MBE: 
MBM_08073
PSCO: 
LY89DRAFT_738559
GLZ: 
GLAREA_07222
ANI: 
AN2998.2
AFM: 
AFUA_3G08650
AOR: 
AOR_1_2438174(AO090005001405)
ANG: 
ANI_1_3132024(An02g12420)
AFV: 
AFLA_086410
ACT: 
ACLA_036640
NFI: 
NFIA_068470
PCS: 
Pc20g03600
PDP: 
PDIP_08760
CIM: 
CIMG_07671
CPW: 
CPC735_042860
PBL: 
PAAG_08349
PBN: 
PADG_04250
URE: 
UREG_07615
ABE: 
ARB_04625
TVE: 
TRV_06219
AJE: 
HCAG_04357
PNO: 
SNOG_01593
PTE: 
PTT_13570
BZE: 
COCCADRAFT_23976
BSC: 
COCSADRAFT_22292
BOR: 
COCMIDRAFT_9531
ZTR: 
MYCGRDRAFT_52059
PFJ: 
MYCFIDRAFT_163981
BCOM: 
BAUCODRAFT_27658
NPA: 
UCRNP2_7472
TML: 
GSTUM_00011854001
SPO: 
SPBC2G2.08(ade9) SPBC839.16
CNE: 
CNG00590
CNB: 
CNBG4190
CGI: 
CGB_G6120C
TMS: 
TREMEDRAFT_32433
TVS: 
TRAVEDRAFT_45346
DSQ: 
DICSQDRAFT_124115
PCO: 
PHACADRAFT_256172
SHS: 
STEHIDRAFT_119085 STEHIDRAFT_151320
HIR: 
HETIRDRAFT_122342
PSQ: 
PUNSTDRAFT_114825
ADL: 
AURDEDRAFT_114390
FME: 
FOMMEDRAFT_127750
GTR: 
GLOTRDRAFT_119984
LBC: 
LACBIDRAFT_187631
MPR: 
MPER_10594
MRR: 
Moror_6556
CCI: 
CC1G_13910
SCM: 
SCHCODRAFT_81176
ABP: 
AGABI1DRAFT118122(AGABI1DRAFT_118122)
ABV: 
AGABI2DRAFT183212(AGABI2DRAFT_183212)
CPUT: 
CONPUDRAFT_49023
SLA: 
SERLADRAFT_454136
WSE: 
WALSEDRAFT_31760
WIC: 
J056_002521
UMA: 
UMAG_10840
PFP: 
PFL1_05629
MGL: 
MGL_2903
PGR: 
PGTG_15310
MLR: 
MELLADRAFT_102871
MBR: 
MONBRDRAFT_7587
SRE: 
PTSG_09498
SMIN: 
v1.2.000751.t1(symbB.v1.2.000751.t1)
AAF: 
AURANDRAFT_5521
PIF: 
PITG_22572
EHX: 
EMIHUDRAFT_462980
TCR: 
511741.50
LMA: 
LMJF_30_2600(FTHS)
LIF: 
LINJ_30_2590(FTHS)
LDO: 
LDBPK_302590
LMI: 
LMXM_29_2600
LBZ: 
LBRM_30_2550(FTHS)
NGR: 
NAEGRDRAFT_77106
ATM: 
ANT_16290(fhs)
ABAT: 
CFX1CAM_0631(fhs)
CAP: 
CLDAP_24240(fhs)
 » show all
TaxonomyKoalaUniProt
Reference
  Authors
Brody LC, Conley M, Cox C, Kirke PN, McKeever MP, Mills JL, Molloy AM, O'Leary VB, Parle-McDermott A, Scott JM, Swanson DA
  Title
A polymorphism, R653Q, in the trifunctional enzyme methylenetetrahydrofolate dehydrogenase/methenyltetrahydrofolate cyclohydrolase/formyltetrahydrofolate synthetase is a maternal genetic risk factor for neural tube defects: report of the Birth Defects Research Group.
  Journal
Am J Hum Genet 71:1207-15 (2002)
DOI:10.1086/344213
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