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

View Ontology Report

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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Genes in Pathway:

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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:6907045
RGD:1547843
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:6907045
RGD:7242561
RGD:70804
G Dhfr dihydrofolate reductase JBrowse link 2 21,931,887 21,958,927 RGD:1298877
RGD:7242561
RGD:6907045
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, phosphoribosylglycinamide synthetase, phosphoribosylaminoimidazole synthetase JBrowse link 11 31,780,477 31,805,728 RGD:1547842
RGD:7242561
RGD:6907045
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 70,952,209 70,986,393 RGD:6907045
G Mthfd1 methylenetetrahydrofolate dehydrogenase, cyclohydrolase and formyltetrahydrofolate synthetase 1 JBrowse link 6 99,282,850 99,350,367 RGD:6907045
RGD:7242561
RGD:1299513
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 4 115,004,433 115,015,965 RGD:1540378
RGD:6907045
G Mthfd2l methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 2-like JBrowse link 14 18,640,274 18,721,298 RGD:6907045
G Mthfr methylenetetrahydrofolate reductase JBrowse link 5 164,844,642 164,864,360 RGD:1535021
RGD:7242561
RGD:6907045
G Mthfs methenyltetrahydrofolate synthetase JBrowse link 8 96,564,877 96,614,386 RGD:6907045
G Mtr 5-methyltetrahydrofolate-homocysteine methyltransferase JBrowse link 17 66,210,444 66,295,014 RGD:6907045
RGD:7242426
RGD:1359026
G Mtrr 5-methyltetrahydrofolate-homocysteine methyltransferase reductase JBrowse link 1 37,743,089 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 5 120,869,141 120,870,270 RGD:6907045
G Sardh sarcosine dehydrogenase JBrowse link 3 5,737,203 5,802,153 RGD:634034
G Shmt1 serine hydroxymethyltransferase 1 JBrowse link 10 47,030,813 47,059,216 RGD:1359815
RGD:7242561
RGD:6907045
RGD:2300383
G Shmt2 serine hydroxymethyltransferase 2 JBrowse link 7 70,824,718 70,829,822 RGD:1359815
RGD:7242561
RGD:6907045
RGD:2300383
G Tyms thymidylate synthetase JBrowse link 9 121,918,875 121,931,564 RGD:6907045
RGD:1545055
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 JBrowse link 5 164,844,642 164,864,360 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
Abortion, SpontaneousDhfr
Abruptio PlacentaeMthfd1
AcidosisAmt
Acute Kidney InjuryMthfr
Adenocarcinoma, ColorectalTyms
AdenomaMtrr
AICAR Transformylase Inosine Monophosphate Cyclohydrolase DeficiencyAtic
Aicar Transformylase/Imp Cyclohydrolase DeficiencyAtic
AlbuminuriaMthfr
AlopeciaMthfr
Alzheimer DiseaseMthfr
Amyotrophic Lateral SclerosisMthfr
Amyotrophic Lateral Sclerosis 1Folh1
AnemiaMthfr , Tyms
Anemia, MegaloblasticDhfr
Anemia, Sickle CellMthfr
AnencephalyMtrr
Aortic Aneurysm, AbdominalMthfr
Aortic CoarctationMthfr
Arakawa Syndrome 2Mtr
Arsenic PoisoningMthfr
Arterial Occlusive DiseasesMthfr
Arthritis, ExperimentalAtic
Arthritis, RheumatoidAtic , Dhfr , Gart , Ggh , Mthfr , Tyms
AsthmaMthfr
AtherosclerosisMthfr
Attention Deficit Disorder with HyperactivityMthfr
Autistic DisorderDhfr , Mthfr , Mtr
Bethlem myopathyFtcd
Bipolar DisorderMthfr
BlindnessAtic
Blood Coagulation Disorders, InheritedMthfr
Brain DiseasesTyms
Brain IschemiaMthfr
Breast NeoplasmsDhfr , Mthfr , Mtr , Shmt1 , Tyms
Budd-Chiari SyndromeMthfr
Burkitt LymphomaFtcd
Carcinoma, HepatocellularGnmt , Mthfd2 , Tyms
Carcinoma, Non-Small-Cell LungShmt2
Carcinoma, Renal CellMthfr
Carcinoma, Squamous CellMthfr , Tyms
Carcinoma, Transitional CellGgh , Mthfr
Cardiac Valvular Defect, DevelopmentalMthfd1
Cardiovascular DiseasesMthfr
Carotid StenosisMthfr
CataractMthfr
Cerebral InfarctionMthfr
Cervical Intraepithelial NeoplasiaMthfr
Chemical and Drug Induced Liver InjuryDhfr , Ggh , Mthfr
CholangiocarcinomaMthfr
Clear-Cell Metastatic Renal Cell CarcinomaMthfr
Cleft LipMthfd1 , Mthfr , Mthfs , Mtr , Tyms
Cleft PalateMthfd1 , Mthfs , Mtr , Tyms
ClubfootMthfr
Colonic NeoplasmsMthfr , Tyms
Colorectal NeoplasmsDhfr , Ggh , Mthfr , Mtrr , Tyms
Combined Oxidative Phosphorylation Deficiency 15Mtfmt
Conotruncal Cardiac DefectsMthfr
Coronary Artery DiseaseMthfr
Coronary DiseaseMthfr
Coronary RestenosisMthfr
Craniofacial AbnormalitiesMthfd1l
Depressive DisorderMthfr
Diabetes Mellitus, ExperimentalMthfr , Mtr
Diabetes Mellitus, Type 1Mthfr
Diabetes Mellitus, Type 2Mthfr , Mtr
Diabetic AngiopathiesMthfr
Diabetic NephropathiesMthfr , Mtrr
Diabetic RetinopathyMthfr
Digestive System NeoplasmsTyms
Dimethylglycine Dehydrogenase DeficiencyDmgdh
Down SyndromeGart , Mthfd1 , Mthfr , Mtrr
Drug EruptionsMthfr
Drug-Related Side Effects and Adverse ReactionsAtic , Dhfr , Mthfr , Mtr , Mtrr , Shmt1 , Tyms
Dystonia 27Ftcd
Endometrial NeoplasmsMthfr
EpilepsyMtr
Eye DiseasesMthfr
Fatty LiverMthfr
Fetal Growth RetardationMthfd1
Folic Acid DeficiencyDhfr
Gastrointestinal DiseasesMthfr , Mtr
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
Head and Neck NeoplasmsTyms
Hearing LossMthfr , Mtr
Hearing Loss, SuddenMthfr , Mtr
Heart Defects, CongenitalMthfr
Heart DiseasesMtr , Mtrr
Heart Septal DefectsMtrr
Hematologic DiseasesMthfr , Mtr
Hematologic NeoplasmsMthfr
Hemophilia AMthfr
HepatitisGnmt
Hepatitis, ChronicGnmt
Hodgkin DiseaseMthfd2
HomocystinuriaMthfr , Mtrr
Homocystinuria-Megaloblastic Anemia due to Defect in Cobalamin Metabolism, CblE Complementation TypeMtrr
HyperammonemiaTyms
Hyperglycinemia, NonketoticAmt
HyperhomocysteinemiaGnmt , Mthfr , Mtr , Mtrr
HypermethioninemiaGnmt
HypersensitivityMthfr
HypertensionMtr
HyperthyroidismMthfr
HypopituitarismMthfr
HypothyroidismMthfr
HypoxiaAldh1l1 , Sardh
Infertility, FemaleDhfr , Mthfr
Infertility, MaleMthfr , Mtr , Mtrr
Inflammatory Bowel Disease 11Mtrr
Inflammatory Bowel DiseasesMthfr
IschemiaMthfr
Kidney DiseasesMthfr
Kidney Failure, ChronicMthfr
Kidney Reperfusion InjuryDhfr
Leigh DiseaseMtfmt
Leigh Syndrome Due To Mitochondrial Complex I DeficiencyMtfmt
Leukemia, Lymphocytic, Chronic, B-CellMthfr
Leukemia, Myelogenous, Chronic, BCR-ABL PositiveMthfr
Leukemia, MyeloidDhfr
Leukemia, Myeloid, AcuteMthfr , Mtrr , Tyms
LeukoencephalopathiesMthfr , Mtr
LeukopeniaMthfr
Liver Cirrhosis, ExperimentalAldh1l1 , Aldh1l2 , Ftcd , Ggh
Liver DiseasesMthfr
Liver NeoplasmsGnmt , Mthfr
Lung NeoplasmsMthfr , Tyms
LymphomaMthfr , Mtr
Lymphoma, FollicularMthfr , Mtr , Tyms
Lymphoma, Large B-Cell, DiffuseMthfr
Lymphoma, Non-HodgkinMthfr , Mtrr , Shmt1 , Tyms
MalnutritionMthfr , Mtr
Mammary Neoplasms, ExperimentalTyms
Maxillofacial AbnormalitiesMthfr
Megaloblastic Anemia due to Dihydrofolate Reductase DeficiencyDhfr
Meniere DiseaseMthfr
MeningiomaMtrr
MeningomyeloceleMthfr
Metabolism, Inborn ErrorsDhfr , Mtr , Mtrr
Methylcobalamin Deficiency, CblG TypeMtr
Methylenetetrahydrofolate Reductase DeficiencyMthfr
Microsatellite InstabilityMthfr
Microvascular AnginaMthfr
Migraine DisordersMthfr
Migraine with AuraMthfr
Mthfr Deficiency, Thermolabile TypeMthfr
MucositisMthfr
Multiple MyelomaMthfr , Mtr , Mtrr , Tyms
Myelodysplastic SyndromesMtrr
Myeloproliferative DisordersMthfr
Myocardial InfarctionMthfr
Myosclerosis, Autosomal RecessiveFtcd
NecrosisMthfr
Neoplasm MetastasisDhfr , Mthfr , Tyms
Neoplasm Recurrence, LocalTyms
NephrosclerosisMthfr
Nervous System DiseasesDhfr , Mthfr , Mtrr , Shmt1 , Tyms
Neural Tube Defect, Folate-SensitiveMthfd1 , Mthfr , Mtr , Mtrr
Neural Tube DefectsMthfd1 , Mthfd1l , Mthfr
Neuroendocrine TumorsTyms
NeutropeniaMthfr
Nonarteritic Anterior Ischemic Optic NeuropathyMthfr
ObesityAldh1l1
Oral Squamous Cell CarcinomaMthfr
OsteonecrosisMthfr
OsteoporosisAtic
OsteosarcomaDhfr , Mthfr
Ovarian NeoplasmsShmt1 , Tyms
Pancreatic NeoplasmsMthfr , Mtr , Mtrr , Tyms
PancytopeniaDhfr
Parkinson DiseaseMtrr
Peripheral Arterial DiseaseMthfr
Precursor Cell Lymphoblastic Leukemia-LymphomaDhfr , Mthfr , Mtr , Mtrr , Tyms
Prostatic NeoplasmsFolh1 , Gnmt , Mthfr , Tyms
Pulmonary EmbolismMthfr
Purine-Pyrimidine Metabolism, Inborn ErrorsAtic
Rectal NeoplasmsMthfr , Tyms
Renal InsufficiencyMthfr
Renal Insufficiency, ChronicMthfr
Renal Vascular DiseasesMthfr
Retinal DiseasesMthfr
Retinal Vein OcclusionMthfr
RetinoblastomaMtr
SarcosinemiaSardh
SchizophreniaMthfr
Scleroatonic Muscular DystrophyFtcd
Sinus Thrombosis, IntracranialMthfr
Skin DiseasesMthfr
Small Cell Lung CarcinomaMthfr
Smith-Magenis SyndromeShmt1
Spinal Cord DiseasesMthfr
Spinal DysraphismMthfd1 , Mthfr , Mtr , Mtrr
SplenomegalyTyms
Stomach NeoplasmsMthfr , Tyms
Stomatitis, AphthousMthfr
StrokeMthfr
Temporomandibular Joint DisordersMtrr
Tetralogy of FallotMthfd1 , Mthfr
ThrombocytopeniaMthfr
ThrombophiliaMthfr
ThrombosisMthfr
Thrombotic MicroangiopathiesMthfr
ThyrotoxicosisMthfr
Tongue NeoplasmsTyms
Transposition of Great VesselsMthfr
Urinary Bladder NeoplasmsGgh , Mthfr , Mtr , Tyms
Uterine Cervical NeoplasmsMthfr
Vascular CalcificationMthfr
Vascular DiseasesMthfr
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
choline metabolic pathwayMtr
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
remethylation pathway of homocysteine metabolism - cobalamin independent, betaine dependentMtr
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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Import into Pathway Studio: