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GeneE
50 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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MTHFR
methylenetetrahydrofolate reductase
Chromosome 1 Β· 1p36.22
NCBI Gene: 4524Ensembl: ENSG00000177000.13HGNC: HGNC:7436UniProt: A0A286YFD0
3,862PubMed Papers
24Diseases
0Drugs
200Pathogenic Variants
RESEARCH IMPACT
Highly StudiedLandmark GeneVariant-Rich
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
methylenetetrahydrofolate reductase [NAD(P)H] activitytetrahydrofolate interconversionprotein bindingprotein-containing complex bindinghomocystinuria due to methylene tetrahydrofolate reductase deficiencyschizophrenianeural tube defects, folate-sensitivethrombophilia due to thrombin defect
✦AI Summary

MTHFR (methylenetetrahydrofolate reductase) catalyzes the conversion of 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate, serving as a critical regulatory link between folate and methionine metabolic cycles 1. This enzyme maintains methionine and homocysteine balance essential for DNA, RNA, and protein synthesis and methylation 2. MTHFR functions as a key regulator of one-carbon metabolism, supporting cellular homeostasis 2. The common C677T polymorphism (p.Ala222Val), carried by approximately half the human population, reduces enzyme activity at body temperature and is the most prevalent cause of elevated homocysteine levels 23. This polymorphism's phenotypic impact depends on dietary folate levels and genetic background 3. Rare missense variants causing MTHFR deficiency represent the most common inherited folate metabolism disorder 3. Clinically, MTHFR polymorphisms associate with multiple conditions: the C677T variant increases ischemic stroke risk 4 and influences intracranial and cerebral hemorrhage susceptibility 5. Associations exist with rheumatoid arthritis 6, congenital heart disease 7, and childhood leukemia, where certain polymorphisms are protective 8. Elevated homocysteine from reduced MTHFR activity represents an independent cardiovascular disease risk factor 2. Vitamin B12 and folate supplementation can reduce plasma homocysteine levels and may prevent disease progression 9.

Sources cited
1
MTHFR catalyzes conversion of 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate for homocysteine remethylation to methionine
PMID: 29891918
2
MTHFR is vital for one-carbon cycle including methionine and folate metabolism; C677T polymorphism causes elevated homocysteine and CVD risk
PMID: 34960114
3
C677T variant (p.Ala222Val) is carried by half of humans; variant effects depend on folinate environment; rare missense variants cause MTHFR deficiency
PMID: 34214447
4
MTHFR C677T polymorphism is significantly associated with increased ischemic stroke risk
PMID: 24140489
5
MTHFR polymorphisms rs1801131 and rs1801133 influence intracranial and cerebral hemorrhage risk
PMID: 33247557
6
MTHFR C677T polymorphism is associated with rheumatoid arthritis susceptibility
PMID: 32170488
7
MTHFR polymorphisms rs1801131 and rs1801133 are associated with congenital heart disease risk
PMID: 30333252
8
MTHFR polymorphisms show associations with leukemia risk, with protective effects in certain populations
PMID: 27966457
9
C677T polymorphism reduces enzyme activity; vitamin B12 and folic acid supplementation reduces homocysteine levels
PMID: 25449138
Disease Associationsβ“˜24
homocystinuria due to methylene tetrahydrofolate reductase deficiencyOpen Targets
0.86Strong
schizophreniaOpen Targets
0.65Moderate
neural tube defects, folate-sensitiveOpen Targets
0.57Moderate
thrombophilia due to thrombin defectOpen Targets
0.54Moderate
Global developmental delayOpen Targets
0.42Moderate
Bilateral tonic-clonic seizureOpen Targets
0.42Moderate
Ischemic strokeOpen Targets
0.40Weak
SeizureOpen Targets
0.37Weak
isolated spina bifidaOpen Targets
0.37Weak
Abnormality of metabolism/homeostasisOpen Targets
0.34Weak
Mental deteriorationOpen Targets
0.34Weak
infantile spasmsOpen Targets
0.33Weak
Delayed speech and language developmentOpen Targets
0.33Weak
Lower limb spasticityOpen Targets
0.33Weak
Secondary microcephalyOpen Targets
0.33Weak
SpasticityOpen Targets
0.33Weak
hypertensionOpen Targets
0.29Weak
Generalized cerebral atrophy/hypoplasiaOpen Targets
0.26Weak
Rare genetic intellectual disabilityOpen Targets
0.26Weak
preeclampsiaOpen Targets
0.24Weak
Homocystinuria due to deficiency of N(5,10)-methylenetetrahydrofolate reductase activityUniProt
Ischemic strokeUniProt
Neural tube defects, folate-sensitiveUniProt
SchizophreniaUniProt
Pathogenic Variants200
NM_005957.5(MTHFR):c.474A>T (p.Gly158=)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive|not provided
β˜…β˜…β˜†β˜†2026β†’ Residue 158
NM_005957.5(MTHFR):c.1013T>C (p.Met338Thr)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive
β˜…β˜…β˜†β˜†2026β†’ Residue 338
NM_005957.5(MTHFR):c.971A>G (p.Asn324Ser)Likely pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency
β˜…β˜…β˜†β˜†2026β†’ Residue 324
NM_005957.5(MTHFR):c.1699C>T (p.Arg567Ter)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|not provided|Neural tube defects, folate-sensitive|Neural tube defects, folate-sensitive;Homocystinuria due to methylene tetrahydrofolate reductase deficiency;Schizophrenia;Thrombophilia due to thrombin defect
β˜…β˜…β˜†β˜†2026β†’ Residue 567
NM_005957.5(MTHFR):c.237-2A>GPathogenic
Neural tube defects, folate-sensitive|Homocystinuria due to methylene tetrahydrofolate reductase deficiency
β˜…β˜…β˜†β˜†2025
NM_005957.5(MTHFR):c.1130G>A (p.Arg377His)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive|Thrombophilia due to thrombin defect;Homocystinuria due to methylene tetrahydrofolate reductase deficiency;Schizophrenia;Neural tube defects, folate-sensitive|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 377
NM_005957.5(MTHFR):c.155G>A (p.Arg52Gln)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|not provided|Thrombophilia due to thrombin defect;Homocystinuria due to methylene tetrahydrofolate reductase deficiency;Schizophrenia;Neural tube defects, folate-sensitive|Neural tube defects, folate-sensitive
β˜…β˜…β˜†β˜†2025β†’ Residue 52
NM_005957.5(MTHFR):c.176G>A (p.Trp59Ter)Likely pathogenic
Neural tube defects, folate-sensitive|Homocystinuria due to methylene tetrahydrofolate reductase deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 59
NM_005957.5(MTHFR):c.1970G>C (p.Ter657Ser)Pathogenic
Intellectual disability|Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive
β˜…β˜…β˜†β˜†2025β†’ Residue 657
NM_005957.5(MTHFR):c.1408G>T (p.Glu470Ter)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 470
NM_005957.5(MTHFR):c.1768del (p.Leu590fs)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive
β˜…β˜…β˜†β˜†2025β†’ Residue 590
NM_005957.5(MTHFR):c.1072C>T (p.Arg358Ter)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive
β˜…β˜…β˜†β˜†2025β†’ Residue 358
NM_005957.5(MTHFR):c.1632+2T>GPathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|not provided|Schizophrenia;Thrombophilia due to thrombin defect;Homocystinuria due to methylene tetrahydrofolate reductase deficiency;Neural tube defects, folate-sensitive|Neural tube defects, folate-sensitive
β˜…β˜…β˜†β˜†2025
NM_005957.5(MTHFR):c.1748G>A (p.Trp583Ter)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 583
NM_005957.5(MTHFR):c.137G>A (p.Arg46Gln)Likely pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive
β˜…β˜…β˜†β˜†2025β†’ Residue 46
NM_005957.5(MTHFR):c.1167-2delPathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive|not provided
β˜…β˜…β˜†β˜†2025
NM_005957.5(MTHFR):c.1121dup (p.Tyr374Ter)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 374
NM_005957.5(MTHFR):c.237-1G>TPathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|not provided
β˜…β˜…β˜†β˜†2025
NM_005957.5(MTHFR):c.337G>A (p.Ala113Thr)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive
β˜…β˜…β˜†β˜†2025β†’ Residue 113
NM_005957.5(MTHFR):c.202C>G (p.Arg68Gly)Pathogenic
Homocystinuria due to methylene tetrahydrofolate reductase deficiency|Neural tube defects, folate-sensitive|Schizophrenia;Thrombophilia due to thrombin defect;Homocystinuria due to methylene tetrahydrofolate reductase deficiency;Neural tube defects, folate-sensitive
β˜…β˜…β˜†β˜†2025β†’ Residue 68
View on ClinVar β†—
Related Genes
GARTProtein interaction100%MTHFD2Protein interaction99%DPYDProtein interaction99%BHMT2Protein interaction98%MMADHCProtein interaction98%GSTP1Protein interaction95%
Tissue Expression6 tissues
Bone Marrow
100%
Heart
89%
Ovary
86%
Lung
79%
Liver
47%
Brain
27%
Gene Interaction Network
Click a node to explore
MTHFRGARTMTHFD2DPYDBHMT2MMADHCGSTP1
PROTEIN STRUCTURE
Preparing viewer…
PDB6FCX Β· 2.50 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.86LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.68 [0.54–0.86]
RankingsWhere MTHFR stands among ~20K protein-coding genes
  • #9of 20,598
    Most Researched3,862 Β· top 1%
  • #337of 5,498
    Most Pathogenic Variants200 Β· top 10%
  • #7,512of 17,882
    Most Constrained (LOEUF)0.86
Genes detectedMTHFR
Sources retrieved50 papers
Response timeβ€”
πŸ“„ Sources
50β–Ό
1
Methylenetetrahydrofolate (MTHFR), the One-Carbon Cycle, and Cardiovascular Risks.
PMID: 34960114
Nutrients Β· 2021
1.00
2
Relationship between MTHFR, MTRR gene polymorphisms and H-type hypertension: a systematic review and meta-analysis.
PMID: 40256868
Ann Hum Biol Β· 2025
0.94
3
Association between MTHFR (677C>T and 1298A>C) polymorphisms and psychiatric disorder: A meta-analysis.
PMID: 35834596
PLoS One Β· 2022
0.92
4
Methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism: epidemiology, metabolism and the associated diseases.
PMID: 25449138
Eur J Med Genet Β· 2015
0.90
5
Association between MTHFR polymorphisms (MTHFR C677T, MTHFR A1298C) and recurrent implantation failure: a systematic review and meta-analysis.
PMID: 33128585
Arch Gynecol Obstet Β· 2021
0.84