FOLATE MEDIATED ONE-CARBON METABOLIC PATHWAY (PW:0000189)

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Description

Tetrahydrofolates (THF) are soluble forms of vitamin B9 that play essential roles in a number of metabolic pathways by mediating the transfer of 1-carbon (1C) units for the de novo synthesis of purines and thymidylates and for the re-methylation of homocysteine (Hcy) to methionine (Met). Humans cannot synthesize folate and must derive it from diet or as a supplement in the form of folic acid (FA). The folate/FA consist of three moieties: a pterin (pteridine) ring, a para aminobenzoyl group (PABA

Pathway Diagram:

Ariadne Genomics Inc. homocysteine ---> methionine homocysteine 5-methyl-THF S-adenosylmethionine methionine Cbl 5-methyl-THF ---> THF 10-formyl-THF ---> de novo purine biosynthetic pathway ADP <--> ATP THF DHF <--> THF 5,10-methylene-THF <--> THF PLP/B6 DHF <--> 5,10-methylene-THF dTMP <--> dUMP 10-formyl-THF <--> THF NADP+ NADPH FAD dUMP dTMP DHF 5,10-methylene-THF ---> 5-methyl-THF formate Dhfr Shmt2 Shmt1 Tyms Atic Gart folate cycle metabolic pathway 5,10-methylene-THF <--> 10-formyl-THF methyl group transfer  to carbon, nitrogen, oxygen or sulfur atoms on proteins, nucleic acids, carbohydrates, lipids, small molecules methionine cycle/metabolic pathway 5,10-methylene-THF DNA synthesis, DNA repair dTMP ---> DNA synthesis, DNA repair Mthfr Mtr Mtrr Mtrr ---> Mtr 10-formyl-THF de novo purine biosynthetic pathway remethylation pathway of homocysteine metabolism - cobalamin dependent remethylation pathway of homocysteine metabolism - cobalamin dependent <--> methionine cycle/metabolic pathway ATP ADP NADPH ---> NADP+ Mthfd1 formate ---> 10-formyl-THF
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folate mediated one-carbon metabolic pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Aldh1l1 aldehyde dehydrogenase 1 family, member L1 JBrowse link 4 123,516,553 123,564,067 RGD:1547843
RGD:6907045
G Aldh1l2 aldehyde dehydrogenase 1 family, member L2 JBrowse link 7 26,375,866 26,425,838 RGD:6907045
G Amt aminomethyltransferase JBrowse link 8 117,068,388 117,078,633 RGD:6907045
G Atic 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase JBrowse link 9 78,862,013 78,882,061 RGD:70804
RGD:6907045
RGD:7242561
G Dhfr dihydrofolate reductase JBrowse link 2 21,931,887 21,958,927 RGD:1298877
RGD:6907045
RGD:7242561
G Dmgdh dimethylglycine dehydrogenase JBrowse link 2 23,289,376 23,370,360 RGD:1547831
G Folh1 folate hydrolase 1 JBrowse link 1 150,323,768 150,395,415 RGD:728645
G Ftcd formimidoyltransferase cyclodeaminase JBrowse link 20 12,806,957 12,820,466 RGD:6907045
G Gart phosphoribosylglycinamide formyltransferase JBrowse link 11 31,780,477 31,805,728 RGD:1547842
RGD:6907045
RGD:7242561
G Ggh gamma-glutamyl hydrolase JBrowse link 5 34,040,258 34,063,369 RGD:69845
G Gnmt glycine N-methyltransferase JBrowse link 9 16,565,274 16,568,626 RGD:1545057
G Mtfmt mitochondrial methionyl-tRNA formyltransferase JBrowse link 8 70,952,173 70,986,393 RGD:6907045
G Mthfd1 methylenetetrahydrofolate dehydrogenase, cyclohydrolase and formyltetrahydrofolate synthetase 1 JBrowse link 6 99,282,850 99,350,367 RGD:1299513
RGD:6907045
RGD:7242561
G Mthfd1l methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like JBrowse link 1 40,529,092 40,719,232 RGD:6907045
G Mthfd2 methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 2, methenyltetrahydrofolate cyclohydrolase JBrowse link 4 115,004,448 115,015,965 RGD:1540378
RGD:6907045
G Mthfd2l methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 2-like JBrowse link 14 18,640,279 18,720,357 RGD:6907045
G Mthfr methylenetetrahydrofolate reductase (NAD(P)H) JBrowse link 5 164,844,653 164,864,360 RGD:1535021
RGD:6907045
RGD:7242561
G Mthfs 5,10-methenyltetrahydrofolate synthetase (5-formyltetrahydrofolate cyclo-ligase) JBrowse link 8 96,564,877 96,614,384 RGD:6907045
G Mtr 5-methyltetrahydrofolate-homocysteine methyltransferase JBrowse link 17 66,210,444 66,295,014 RGD:1359026
RGD:6907045
RGD:7242426
G Mtrr 5-methyltetrahydrofolate-homocysteine methyltransferase reductase JBrowse link 1 37,743,100 37,774,485 RGD:7242426
G RGD1564040 similar to methylenetetrahydrofolate dehydrogenase (NAD) (EC 1.5.1.15)/methenyltetrahydrofolate cyclohydrolase (EC 3.5.4.9) precursor JBrowse link 5 120,869,141 120,870,188 RGD:6907045
G Sardh sarcosine dehydrogenase JBrowse link 3 5,737,202 5,802,146 RGD:634034
G Shmt1 serine hydroxymethyltransferase 1 JBrowse link 10 47,030,812 47,059,216 RGD:1359815
RGD:2300383
RGD:6907045
RGD:7242561
G Shmt2 serine hydroxymethyltransferase 2 JBrowse link 7 70,824,719 70,829,824 RGD:1359815
RGD:2300383
RGD:6907045
RGD:7242561
G Tyms thymidylate synthetase JBrowse link 9 121,918,875 121,931,564 RGD:1545055
RGD:6907045
RGD:7242561
altered folate mediated one-carbon metabolic pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Mthfd1 methylenetetrahydrofolate dehydrogenase, cyclohydrolase and formyltetrahydrofolate synthetase 1 JBrowse link 6 99,282,850 99,350,367 RGD:7242562
G Mthfr methylenetetrahydrofolate reductase (NAD(P)H) JBrowse link 5 164,844,653 164,864,360 RGD:7242562
G Mtr 5-methyltetrahydrofolate-homocysteine methyltransferase JBrowse link 17 66,210,444 66,295,014 RGD:7242562

Altered Pathway:

Pathway Gene Annotations

Disease Annotations Associated with Genes in the folate mediated one-carbon metabolic pathway
Disease TermsGene Symbols
Abortion, HabitualMthfr
AcidosisAmt
Adenocarcinoma, ColorectalTyms
Aicar Transformylase/Imp Cyclohydrolase DeficiencyAtic
AlbuminuriaMthfr
Amyotrophic Lateral Sclerosis 1Folh1
AnemiaMthfr
Anemia, Sickle CellMthfr
Aortic Aneurysm, AbdominalMthfr
Aortic CoarctationMthfr
Arakawa Syndrome 2Mtr
Arterial Occlusive DiseasesMthfr
Arthritis, ExperimentalAtic
Arthritis, RheumatoidMthfr , Tyms
AsthmaMthfr
AtherosclerosisMthfr
Autistic DisorderMtr
Breast NeoplasmsShmt1
Budd-Chiari SyndromeMthfr
Carcinoma, HepatocellularMthfd2
Carcinoma, Renal CellMthfr
Carcinoma, Squamous CellMthfr
Carcinoma, Transitional CellMthfr
Cardiovascular DiseasesMthfr
Carotid StenosisMthfr
CataractMthfr
Cerebral InfarctionMthfr
CholangiocarcinomaMthfr
Clear-Cell Metastatic Renal Cell CarcinomaMthfr
Colitis, UlcerativeMtrr
Colorectal NeoplasmsTyms
Combined Oxidative Phosphorylation Deficiency 15Mtfmt
Coronary Artery DiseaseMthfr
Diabetes Mellitus, ExperimentalMthfr , Mtr
Diabetes Mellitus, Type 1Mthfr
Diabetes Mellitus, Type 2Mthfr , Mtr
Diabetic NephropathiesMthfr , Mtrr
Diabetic RetinopathyMthfr
Dimethylglycine Dehydrogenase DeficiencyDmgdh
Down SyndromeGart , Mtrr
EpilepsyMtr
Eye DiseasesMthfr
Fatty LiverMthfr
Gastrointestinal Stromal TumorsDhfr , Mthfr , Mtr , Mtrr , Shmt1
Genetic Diseases, InbornMtr , Mtrr
Glaucoma, Angle-ClosureMthfr
Glaucoma, Open-AngleMthfr
GlomerulonephritisMthfr
Glomerulonephritis, IGAMtr
Glutamate Formiminotransferase DeficiencyFtcd
Graft vs Host DiseaseMthfr
Graves DiseaseMthfr
Hearing LossMthfr , Mtr
Hearing Loss, SuddenMthfr , Mtr
Heart DiseasesMtr , Mtrr
Heart Septal DefectsMtrr
Hematologic NeoplasmsMthfr
Hemophilia AMthfr
HomocysteinemiaMthfr
HomocystinuriaMthfr , Mtrr
Homocystinuria-Megaloblastic Anemia due to Defect in Cobalamin Metabolism, CblE Complementation TypeMtrr
Hyperglycinemia, NonketoticAmt
HyperhomocysteinemiaMthfr , Mtr
HypermethioninemiaGnmt
HypertensionMtr
HyperthyroidismMthfr
HypopituitarismMthfr
HypothyroidismMthfr
Inflammatory Bowel DiseasesMthfr
IschemiaMthfr
Kidney Failure, ChronicMthfr
Kidney Reperfusion InjuryDhfr
Leigh DiseaseMtfmt
Leigh Syndrome Due To Mitochondrial Complex I DeficiencyMtfmt
Leukemia, Myelogenous, Chronic, BCR-ABL PositiveMthfr
Leukemia, MyeloidDhfr
Leukemia, Myeloid, AcuteMthfr , Tyms
Liver NeoplasmsMthfr
Lymphoma, Large B-Cell, DiffuseMthfr
Mammary Neoplasms, ExperimentalTyms
Megaloblastic Anemia due to Dihydrofolate Reductase DeficiencyDhfr
Meniere DiseaseMthfr
MeningiomaMtrr
Methylcobalamin Deficiency, CblG TypeMtr
Methylenetetrahydrofolate Reductase DeficiencyMthfr
Microvascular AnginaMthfr
Migraine DisordersMthfr
Migraine with AuraMthfr
Mthfr Deficiency, Thermolabile TypeMthfr
Multiple MyelomaMthfr , Tyms
Myeloproliferative DisordersMthfr
Myocardial InfarctionMthfr
Neoplasm MetastasisDhfr , Tyms
NephrosclerosisMthfr
Neural Tube Defect, Folate-SensitiveMthfr , Mtr , Mtrr
Neural Tube DefectsMthfd1 , Mthfr
Nonarteritic Anterior Ischemic Optic NeuropathyMthfr
Oral Squamous Cell CarcinomaMthfr
OsteonecrosisMthfr
OsteosarcomaMthfr
Ovarian NeoplasmsShmt1
Pancreatic NeoplasmsMthfr , Mtr , Mtrr
Parkinson DiseaseMtrr
Peripheral Arterial DiseaseMthfr
Precursor Cell Lymphoblastic Leukemia-LymphomaDhfr , Mthfr , Tyms
Prostatic NeoplasmsMthfr
Purine-Pyrimidine Metabolism, Inborn ErrorsAtic
Rectal NeoplasmsMthfr
Renal InsufficiencyMthfr
Renal Insufficiency, ChronicMthfr
Retinal DiseasesMthfr
Retinal Vein OcclusionMthfr
RetinoblastomaMtr
SarcosinemiaSardh
SchizophreniaMthfr
Small Cell Lung CarcinomaMthfr
Spinal DysraphismMthfd1 , Mtr , Mtrr
SplenomegalyTyms
Stomatitis, AphthousMthfr
StrokeMthfr
Temporomandibular Joint DisordersMtrr
ThrombocytopeniaMthfr
ThrombophiliaMthfr
ThrombosisMthfr
Thrombotic MicroangiopathiesMthfr
Urinary Bladder NeoplasmsMthfr , Mtr , Tyms
Vascular CalcificationMthfr
Venous ThromboembolismMthfr
Venous ThrombosisMthfr
Vitamin B 12 DeficiencyMtr
Wet Macular DegenerationMthfr
Wilms TumorMthfr
Pathway Annotations Associated with Genes in the folate mediated one-carbon metabolic pathway
Pathway TermsGene Symbols
adenine phoshoribosyltransferase deficiency pathwayAtic , Gart
adenosine monophosphate deaminase deficiency pathwayAtic , Gart
adenylosuccinate lyase deficiency pathwayAtic , Gart
AICA-ribosuria pathwayAtic , Gart
altered folate cycle metabolic pathwayAldh1l1 , Dhfr , Mthfd1 , Mthfr , Mtr , Mtrr
altered folate mediated one-carbon metabolic pathwayMthfd1 , Mthfr , Mtr
aminoacyl-tRNA biosynthetic pathwayMtfmt
azathioprine pharmacodynamics pathwayAtic , Gart
beta-ureidopropionase deficiency pathwayTyms
capecitabine pharmacodynamics pathwayTyms
capecitabine pharmacokinetics pathwayTyms
carnitine biosynthetic pathwayShmt1
cyanoamino acid metabolic pathwayShmt1 , Shmt2
cysteine and methionine metabolic pathwayMtr
de novo purine biosynthetic pathwayAtic , Gart
dihydropyrimidinase deficiency pathwayTyms
dihydropyrimidine dehydrogenase deficiency pathwayAmt , Dmgdh , Gnmt , Sardh , Shmt1 , Shmt2
dimethylglycine dehydrogenase deficiency pathwayAmt , Dmgdh , Gnmt , Sardh , Shmt1 , Shmt2
dopa responsive dystonia disease pathwayDhfr
folate cycle metabolic pathwayAldh1l1 , Amt , Dhfr , Dmgdh , Folh1 , Ggh , Mthfd1 , Mthfd1l , Mthfd2 , Mthfd2l , Mthfr , Mtr , Mtrr , Sardh , Shmt1 , Shmt2
folate mediated one-carbon metabolic pathwayAldh1l1 , Aldh1l2 , Amt , Atic , Dhfr , Dmgdh , Folh1 , Ftcd , Gart , Ggh , Gnmt , Mtfmt , Mthfd1 , Mthfd1l , Mthfd2 , Mthfd2l , Mthfr , Mthfs , Mtr , Mtrr , RGD1564040 , Sardh , Shmt1 , Shmt2 , Tyms
folate metabolic pathwayAldh1l1 , Atic , Dhfr , Dmgdh , Folh1 , Ftcd , Gart , Ggh , Gnmt , Mtfmt , Mthfd1 , Mthfd1l , Mthfd2 , Mthfr , Mthfs , Mtr , Mtrr , Sardh , Shmt1 , Shmt2 , Tyms
gemcitabine pharmacodynamics pathwayTyms
gemcitabine pharmacokinetics pathwayTyms
glycine metabolic pathwayShmt1 , Shmt2
glycine N-methyltransferase deficiency pathwayMtfmt , Mthfr , Mtr , Shmt1
glycine, serine and threonine metabolic pathwayAmt , Dmgdh , Gnmt , Sardh , Shmt1 , Shmt2
hereditary folate malabsorption disease pathwayAldh1l1 , Dhfr , Ftcd , Ggh , Mtfmt , Mthfd1 , Mthfd1l , Mthfd2 , Mthfr , Mthfs
histidine metabolic pathwayFtcd
histidinemia disease pathwayFtcd
homocysteine metabolic pathwayGnmt , Mthfr
homocystinuria disease pathwayMtfmt , Mthfr , Mtr , Shmt1
hypermethioninemia disease pathwayMtfmt , Mthfr , Mtr , Shmt1
Kelley-Seegmiller syndrome pathwayAtic , Gart
Lesch-Nyhan syndrome pathwayAtic , Gart
mercaptopurine pharmacodynamics pathwayAtic , Gart
methionine adenosyltransferase deficiency pathwayMtfmt , Mthfr , Mtr , Shmt1
methionine cycle/metabolic pathwayGnmt , Mtfmt , Mthfr , Mtr , Mtrr , Shmt1
methotrexate pharmacodynamics pathwayAldh1l1 , Dhfr , Ftcd , Ggh , Mtfmt , Mthfd1 , Mthfd1l , Mthfd2 , Mthfr , Mthfs
methylenetetrahydrofolate reductase deficiency pathwayMtfmt , Mthfr , Mtr , Shmt1
mitochondrial neurogastrointestinal encephalopathy syndrome pathwayTyms
molybdenum cofactor deficiency pathwayAtic , Gart
nonketotic hyperglycinemia disease pathwayAmt , Dmgdh , Gnmt , Sardh , Shmt1 , Shmt2
Nonketotic hyperglycinemia disease pathwayAmt , Dmgdh , Gnmt , Sardh , Shmt1 , Shmt2
oroticaciduria 1 disease pathwayTyms
purine metabolic pathwayAtic , Gart
purine nucleoside phosphorylase deficiency pathwayAtic , Gart
pyrimidine metabolic pathwayDhfr , Tyms
remethylation pathway of homocysteine metabolism - cobalamin dependentMthfr , Mtr , Mtrr
sarcosinemia disease pathwayAmt , Dmgdh , Gnmt , Sardh , Shmt1 , Shmt2
Segawa syndrome pathwayDhfr
selenoamino acid metabolic pathwayMtr
tioguanine pharmacodynamics pathwayAtic , Gart
xanthinuria disease pathwayAtic , Gart
xanthinuria disease pathway, type IAtic , Gart
xanthinuria disease pathway, type IIAtic , Gart

References Associated with the folate mediated one-carbon metabolic pathway:

Ontology Path Diagram:

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