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
GO TO:

Genes in Pathway:

show annotations for term's descendants       view all columns           Sort by:
 
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:7242561
RGD:1545055
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
abdominal aortic aneurysmMthfr
Abortion, HabitualMthfr
Abortion, SpontaneousDhfr
AcidosesAmt
acute kidney failureMthfr
acute myeloid leukemiaMthfr , Mtrr , Tyms
adenomaMtrr
AICAR Transformylase Inosine Monophosphate Cyclohydrolase DeficiencyAtic
AlbuminuriaMthfr
allergic hypersensitivity diseaseMthfr
alopeciaMthfr
Alzheimer's diseaseMthfr
amyotrophic lateral sclerosisMthfr
amyotrophic lateral sclerosis type 1Folh1
anemiaMthfr , Tyms
anencephalyMtrr
angle-closure glaucomaMthfr
Animal Disease ModelsGart , Shmt1 , Tyms
Aortic CoarctationMthfr
aphthous stomatitisMthfr
Arakawa Syndrome 2Mtr
Arsenic PoisoningMthfr
Arterial Occlusive DiseasesMthfr
asthmaMthfr
atherosclerosisMthfr
attention deficit hyperactivity disorderMthfr
autistic disorderDhfr , Ftcd , Mthfr , Mtr , Shmt1 , Tyms
Axenfeld-Rieger syndrome type 3Ftcd
B-Cell Chronic Lymphocytic LeukemiaMthfr
Bethlem myopathyFtcd
bipolar disorderMthfr
blindnessAtic
brain diseaseTyms
brain ischemiaMthfr
breast cancerShmt1
Breast NeoplasmsDhfr , Mthfr , Mtr , Shmt1 , Tyms
Budd-Chiari syndromeMthfr
Burkitt lymphomaFtcd
cardiovascular system diseaseMthfr
carotid stenosisMthfr
cataractMthfr
catecholaminergic polymorphic ventricular tachycardiaMtr
cerebral infarctionMthfr
cervix uteri carcinoma in situMthfr
Chemical and Drug Induced Liver InjuryAldh1l2 , Dhfr , Ggh , Mthfr
cholangiocarcinomaMthfr
Chronic HepatitisGnmt
chronic kidney diseaseMthfr
chronic myeloid leukemiaMthfr
clear cell renal cell carcinomaMthfr
cleft lipMthfd1 , Mthfr , Mthfs , Mtr , Tyms
cleft palateMthfd1 , Mthfs , Mtr , Tyms
clubfootMthfr
colon carcinomaTyms
Colonic NeoplasmsMthfr , Tyms
colorectal adenocarcinomaTyms
Colorectal NeoplasmsDhfr , Ggh , Mthfr , Mtrr , Tyms
COMBINED IMMUNODEFICIENCY AND MEGALOBLASTIC ANEMIA WITH OR WITHOUT HYPERHOMOCYSTEINEMIAMthfd1
combined oxidative phosphorylation deficiencyMtfmt
Combined Oxidative Phosphorylation Deficiency 15Mtfmt
congenital heart diseaseMthfr
Conotruncal Cardiac DefectsMthfr
coronary artery diseaseMthfr
Coronary DiseaseMthfr
coronary restenosisMthfr
Craniofacial AbnormalitiesMthfd1l
Crohn's diseaseMthfr
Developmental Cardiac Valvular DefectMthfd1
dextro-looped transposition of the great arteriesMthfr
Diabetes Mellitus, Experimental Mthfr , Mtr
diabetic angiopathyMthfr
Diabetic NephropathiesMthfr , Mtrr
diabetic retinopathyMthfr
diffuse large B-cell lymphomaMthfr
Digestive System NeoplasmsTyms
dilated cardiomyopathy 1AAMtr
Dimethylglycine Dehydrogenase DeficiencyDmgdh
Down syndromeGart , Mthfd1 , Mthfr , Mtrr
Drug EruptionsMthfr
Drug-Related Side Effects and Adverse ReactionsAtic , Dhfr , Mthfr , Mtr , Mtrr , Shmt1 , Tyms
DwarfismMtfmt
dystonia 27Ftcd
end stage renal failureMthfr
endometrial carcinomaDhfr
Endometrial NeoplasmsMthfr
epilepsyMtr
Experimental ArthritisAtic
Experimental Liver CirrhosisAldh1l1 , Aldh1l2 , Ftcd , Ggh
eye diseaseMthfr
factor VIII deficiencyMthfr
fatty liver diseaseMthfr
Female InfertilityDhfr , Mthfr
Fetal Growth RetardationMthfd1
folic acid deficiency anemiaDhfr
follicular lymphomaMthfr , Mtr , Tyms
Gastrointestinal DiseasesMthfr , Mtr
gastrointestinal stromal tumorDhfr , Mthfr , Mtr , Mtrr , Shmt1
genetic diseaseAmt , Mtr , Mtrr
glomerulonephritisMthfr
Glutamate Formiminotransferase DeficiencyFtcd
glycine encephalopathyAmt
glycine N-methyltransferase deficiencyGnmt
Graft vs Host DiseaseMthfr
Graves' diseaseMthfr
Head and Neck NeoplasmsTyms
Hearing LossMthfr , Mtr
Hearing Loss, SuddenMthfr , Mtr
heart diseaseMtr , Mtrr
heart septal defectMtrr
Hematologic NeoplasmsMthfr
hematopoietic system diseaseMthfr , Mtr
hepatitisGnmt
hepatocellular carcinomaGnmt , Mthfd2 , Tyms
Hodgkin's lymphomaMthfd2
homocystinuriaMthfr , Mtrr
Homocystinuria-Megaloblastic Anemia due to Defect in Cobalamin Metabolism, CblE Complementation TypeMtrr
HyperammonemiaTyms
hyperhomocysteinemiaGnmt , Mthfr , Mtr , Mtrr
hypermethioninemiaGnmt
hypertensionMtr
hyperthyroidismMthfr
hypopituitarismMthfr
hypothyroidismMthfr
HypoxiaAldh1l1 , Sardh
IgA glomerulonephritisMtr
inflammatory bowel diseaseMthfr
inherited blood coagulation diseaseMthfr
inherited metabolic disorderDhfr , Mtr , Mtrr
intracranial sinus thrombosisMthfr
ischemiaMthfr
kidney diseaseMthfr
kidney failureMthfr
Kidney Reperfusion InjuryDhfr
Kuhnt-Junius degenerationMthfr
Leigh diseaseMtfmt
Leigh Syndrome Due To Mitochondrial Complex I DeficiencyMtfmt
LeukoencephalopathiesMthfr , Mtr
leukopeniaMthfr
liver benign neoplasmGnmt , Mthfr
liver diseaseMthfr
Lung NeoplasmsMthfr , Tyms
lung small cell carcinomaMthfr
lymphomaMthfr , Mtr
male infertilityMthfr , Mtr , Mtrr
MalnutritionMthfr , Mtr
Mammary Neoplasms, ExperimentalTyms
Maxillofacial AbnormalitiesMthfr
megaloblastic anemiaDhfr
Megaloblastic Anemia due to Dihydrofolate Reductase DeficiencyDhfr
Meniere's diseaseMthfr
meningiomaMtrr
mental depressionMthfr
Methylenetetrahydrofolate Reductase DeficiencyMthfr
methylmalonic aciduria and homocystinuria type cblGMtr
Microsatellite InstabilityMthfr
Microvascular AnginaMthfr
migraineMthfr
migraine with auraMthfr
Mitochondrial Complex I Deficiency, Nuclear Type 27Mtfmt
mitochondrial metabolism diseaseMtfmt
Mthfr Deficiency, Thermolabile TypeMthfr
mucositisMthfr
multiple myelomaMthfr , Mtr , Mtrr , Tyms
myelodysplastic syndromeMtrr
myeloid leukemiaDhfr
myeloid neoplasmMthfr
myelomeningoceleMthfr
myocardial infarctionMthfr
Myosclerosis, Autosomal RecessiveFtcd
NecrosisMthfr
nemaline myopathy 6Mtfmt
Neoplasm MetastasisDhfr , Mthfr , Tyms
Neoplasm Recurrence, LocalTyms
nephroblastomaMthfr
nephrosclerosisMthfr
nervous system diseaseDhfr , Mthfr , Mtrr , Shmt1 , Tyms
neural tube defectMthfd1 , Mthfd1l , Mthfr , Mtr , Mtrr
Neural Tube Defect, Folate-SensitiveMthfd1 , Mthfr , Mtr , Mtrr
neuroendocrine tumorTyms
neutropeniaMthfr
non-arteritic anterior ischemic optic neuropathyMthfr
non-Hodgkin lymphomaMthfr , Mtrr , Shmt1 , Tyms
non-small cell lung carcinomaShmt2
obesityAldh1l1
open-angle glaucomaMthfr
oral squamous cell carcinomaMthfr
osteonecrosisMthfr
osteoporosisAtic
osteosarcomaDhfr , Mthfr
ovarian cancerShmt1
Ovarian NeoplasmsTyms
pancreatic cancerMthfr , Mtr , Mtrr , Tyms
pancytopeniaDhfr
Parkinson's diseaseMtrr
peripheral artery diseaseMthfr
placental abruptionMthfd1
Potocki-Lupski syndromeShmt1
precursor lymphoblastic lymphoma/leukemiaDhfr , Mthfr , Mtr , Mtrr , Tyms
prostate cancerMthfr
Prostatic NeoplasmsFolh1 , Gnmt , Mthfr , Tyms
pulmonary embolismMthfr
purine-pyrimidine metabolic disorderAtic
rectal neoplasmMthfr , Tyms
renal artery diseaseMthfr
renal cell carcinomaMthfr
retinal diseaseMthfr
retinal vein occlusionMthfr
retinoblastomaMtr
rheumatoid arthritisAtic , Dhfr , Gart , Ggh , Mthfr , Tyms
SarcosinemiaSardh
schizophreniaMthfr
sickle cell anemiaMthfr
skin diseaseMthfr
spina bifidaMthfd1 , Mthfr , Mtr , Mtrr
spinal cord diseaseMthfr
SplenomegalyTyms
squamous cell carcinomaMthfr , Tyms
stomach cancerMthfr
Stomach NeoplasmsMthfr , Tyms
StrokeMthfr
Temporomandibular Joint DisordersMtrr
tetralogy of FallotMthfd1 , Mthfr
thrombocytopeniaMthfr
thrombophiliaMthfr
thrombosisMthfr
Thrombotic MicroangiopathiesMthfr
thyrotoxicosisMthfr
Tongue NeoplasmsTyms
transitional cell carcinomaGgh , Mthfr
type 1 diabetes mellitusMthfr
type 2 diabetes mellitusMthfr , Mtr
ulcerative colitisMtrr
urinary bladder cancerGgh , Mthfr , Mtr , Tyms
Uterine Cervical NeoplasmsMthfr
Vascular CalcificationMthfr
vascular diseaseMthfr
Venous ThromboembolismMthfr
Venous ThrombosisMthfr
vitamin B12 deficiencyMtr
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 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 pathwayAldh1l1 , Dhfr , Ftcd , Ggh , Mtfmt , Mthfd1 , Mthfd1l , Mthfd2 , Mthfr , Mthfs
histidine metabolic pathwayFtcd
histidinemia pathwayFtcd
homocysteine metabolic pathwayGnmt , Mthfr
homocystinuria pathwayMtfmt , Mthfr , Mtr , Shmt1
hypermethioninemia pathwayMtfmt , Mthfr , Mtr , Shmt1
hypermethioninemia pathwayMtfmt , Mthfr , Mtr , Shmt1
Kelley-Seegmiller syndrome pathwayAtic , Gart
Lesch-Nyhan syndrome pathwayAtic , Gart
mercaptopurine pharmacodynamics pathwayAtic , Gart
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 pathwayAmt , Dmgdh , Gnmt , Sardh , Shmt1 , Shmt2
orotic aciduria 1 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 pathwayAmt , Dmgdh , Gnmt , Sardh , Shmt1 , Shmt2
Segawa syndrome pathwayDhfr
selenoamino acid metabolic pathwayMtr
tioguanine pharmacodynamics pathwayAtic , Gart
xanthinuria type I pathwayAtic , Gart
xanthinuria pathwayAtic , Gart
xanthinuria type II pathwayAtic , Gart

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

Ontology Path Diagram:

paths to the root

Import into Pathway Studio: