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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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CYP2R1
cytochrome P450 family 2 subfamily R member 1
Chromosome 11 Β· 11p15.2
NCBI Gene: 120227Ensembl: ENSG00000186104.11HGNC: HGNC:20580UniProt: E9PJT9
120PubMed Papers
21Diseases
0Drugs
30Pathogenic Variants
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
iron ion bindingheme bindingvitamin D3 25-hydroxylase activityprotein homodimerization activityHypocalcemic vitamin D-dependent ricketsvitamin D deficiencyvitamin deficiency disorderrickets
✦AI Summary

CYP2R1 encodes a cytochrome P450 monooxygenase that functions as the principal hepatic 25-hydroxylase in vitamin D metabolism 12. The enzyme catalyzes hydroxylation at the C-25 position of both vitamin D2 and D3, converting parent vitamin D to 25-hydroxyvitamin D (25(OH)D), the major circulating biomarker of vitamin D status 3. CYP2R1 also metabolizes vitamin D analogs including doxercalciferol and alfacalcidol. Mechanistically, the enzyme uses molecular oxygen to insert one atom into substrate while reducing the second to water, with electrons supplied by NADPH via cytochrome P450 reductase 2. Genetic variants in CYP2R1, particularly the rs10741657 polymorphism, significantly influence circulating 25(OH)D levels across populations 4. The G allele associates with reduced 25(OH)D levels and increased vitamin D deficiency risk 4. Loss-of-function CYP2R1 mutations cause vitamin D-dependent rickets type 1B (VDDR1B), a rare inherited disorder characterized by impaired vitamin D activation and rickets despite normal kidney function 3. CYP2R1 polymorphisms are also associated with hypertension risk, particularly when combined with obesity 5, and with coronary artery disease susceptibility 6. These findings underscore CYP2R1's critical role in vitamin D homeostasis and its importance as a genetic modifier of vitamin D-related disease risk.

Sources cited
1
CYP2R1 identified as the most important 25-hydroxylase enzyme in vitamin D metabolism
PMID: 26681795
2
CYP2R1 catalyzes conversion of vitamin D to 25-hydroxyvitamin D; mechanism uses molecular oxygen and NADPH via cytochrome P450 reductase
PMID: 24529992
3
CYP2R1 is principal hepatic 25-hydroxylase; mutations cause vitamin D-dependent rickets type 1B (VDDR1B)
PMID: 27473561
4
CYP2R1 rs10741657 GG genotype associates with lower 25(OH)D levels and increased vitamin D deficiency risk across populations
PMID: 30120973
5
CYP2R1 rs12794714 polymorphism associates with hypertension risk, especially in combination with obesity
PMID: 34906413
6
CYP2R1 rs1562902 C>T variants associated with increased coronary artery disease risk and vitamin D deficiency
PMID: 39600283
Disease Associationsβ“˜21
Hypocalcemic vitamin D-dependent ricketsOpen Targets
0.75Strong
vitamin D deficiencyOpen Targets
0.53Moderate
vitamin deficiency disorderOpen Targets
0.50Moderate
ricketsOpen Targets
0.38Weak
COVID-19Open Targets
0.28Weak
vitamin D-dependent rickets, type 1Open Targets
0.27Weak
hypertrophic cardiomyopathyOpen Targets
0.20Weak
Vogt-Koyanagi-Harada diseaseOpen Targets
0.19Weak
squamous cell carcinomaOpen Targets
0.17Weak
Low serum calcitriolOpen Targets
0.11Weak
amyloidosisOpen Targets
0.09Suggestive
type 1 diabetes mellitusOpen Targets
0.08Suggestive
obesityOpen Targets
0.08Suggestive
coronary artery diseaseOpen Targets
0.08Suggestive
colorectal carcinomaOpen Targets
0.08Suggestive
migraine disorderOpen Targets
0.07Suggestive
myeloid sarcomaOpen Targets
0.07Suggestive
ocular hypotensionOpen Targets
0.06Suggestive
poisoningOpen Targets
0.06Suggestive
hepatocellular carcinomaOpen Targets
0.05Suggestive
Rickets vitamin D-dependent 1BUniProt
Pathogenic Variants30
NM_024514.5(CYP2R1):c.219C>A (p.Tyr73Ter)Pathogenic
not provided|Vitamin D hydroxylation-deficient rickets, type 1B
β˜…β˜…β˜†β˜†2025β†’ Residue 73
NM_024514.5(CYP2R1):c.1120dup (p.Ile374fs)Pathogenic
not provided|Vitamin D hydroxylation-deficient rickets, type 1B
β˜…β˜…β˜†β˜†2025β†’ Residue 374
NM_024514.5(CYP2R1):c.296T>C (p.Leu99Pro)Pathogenic
Vitamin D hydroxylation-deficient rickets, type 1B|not provided|Vitamin D-dependent rickets, type 1|CYP2R1-related disorder|Hypophosphataemia or rickets
β˜…β˜…β˜†β˜†2025β†’ Residue 99
NM_024514.5(CYP2R1):c.628_629del (p.Met210fs)Pathogenic
not provided|Vitamin D hydroxylation-deficient rickets, type 1B
β˜…β˜…β˜†β˜†2025β†’ Residue 210
NM_024514.5(CYP2R1):c.31_40dup (p.Leu14fs)Pathogenic
Vitamin D hydroxylation-deficient rickets, type 1B|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 14
NM_024514.5(CYP2R1):c.367+1G>APathogenic
Vitamin D hydroxylation-deficient rickets, type 1B|not provided
β˜…β˜…β˜†β˜†2024
NM_024514.5(CYP2R1):c.433C>T (p.Arg145Ter)Pathogenic
not provided|Vitamin D hydroxylation-deficient rickets, type 1B|CYP2R1-related disorder
β˜…β˜…β˜†β˜†2024β†’ Residue 145
NM_024514.5(CYP2R1):c.289G>T (p.Glu97Ter)Pathogenic
not provided|Vitamin D hydroxylation-deficient rickets, type 1B
β˜…β˜…β˜†β˜†2024β†’ Residue 97
NM_024514.5(CYP2R1):c.595C>T (p.Arg199Ter)Pathogenic
Vitamin D hydroxylation-deficient rickets, type 1B|not provided
β˜…β˜…β˜†β˜†2022β†’ Residue 199
NM_024514.5(CYP2R1):c.768dup (p.Leu257fs)Pathogenic
Vitamin D hydroxylation-deficient rickets, type 1B|not provided|CYP2R1-related disorder
β˜…β˜…β˜†β˜†2022β†’ Residue 257
NM_024514.5(CYP2R1):c.254C>G (p.Ser85Ter)Pathogenic
not provided
β˜…β˜†β˜†β˜†2026β†’ Residue 85
NM_024514.5(CYP2R1):c.15G>A (p.Trp5Ter)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 5
NM_024514.5(CYP2R1):c.391C>T (p.Arg131Ter)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 131
NM_024514.5(CYP2R1):c.88del (p.Leu30fs)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 30
NM_024514.5(CYP2R1):c.121_122del (p.Pro41fs)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 41
NM_024514.5(CYP2R1):c.135del (p.Leu46fs)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 46
NM_024514.5(CYP2R1):c.41_50del (p.Leu14fs)Pathogenic
not provided
β˜…β˜†β˜†β˜†2024β†’ Residue 14
NM_024514.5(CYP2R1):c.726dup (p.His243fs)Likely pathogenic
Vitamin D hydroxylation-deficient rickets, type 1B
β˜…β˜†β˜†β˜†2024β†’ Residue 243
NM_024514.5(CYP2R1):c.412C>T (p.Arg138Ter)Likely pathogenic
Vitamin D hydroxylation-deficient rickets, type 1B
β˜…β˜†β˜†β˜†2024β†’ Residue 138
NM_024514.5(CYP2R1):c.1000+1G>ALikely pathogenic
Vitamin D hydroxylation-deficient rickets, type 1B
β˜…β˜†β˜†β˜†2024
View on ClinVar β†—
Related Genes
DHCR7Protein interaction96%VDRProtein interaction96%NADSYN1Protein interaction96%CYP27A1Protein interaction75%AMDHD1Protein interaction74%CYP24A1Protein interaction71%
Tissue Expression6 tissues
Liver
100%
Lung
96%
Bone Marrow
86%
Ovary
70%
Heart
64%
Brain
30%
Gene Interaction Network
Click a node to explore
CYP2R1DHCR7VDRNADSYN1CYP27A1AMDHD1CYP24A1
PROTEIN STRUCTURE
Preparing viewer…
PDB3CZH Β· 2.30 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
1.11LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.86 [0.66–1.11]
RankingsWhere CYP2R1 stands among ~20K protein-coding genes
  • #3,915of 20,598
    Most Researched120 Β· top quartile
  • #1,800of 5,498
    Most Pathogenic Variants30
  • #11,417of 17,882
    Most Constrained (LOEUF)1.11
Genes detectedCYP2R1
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Vitamin D: Metabolism, Molecular Mechanism of Action, and Pleiotropic Effects.
PMID: 26681795
Physiol Rev Β· 2016
1.00
2
An Update on Vitamin D Metabolism.
PMID: 32911795
Int J Mol Sci Β· 2020
0.90
3
Vitamin D metabolism, mechanism of action, and clinical applications.
PMID: 24529992
Chem Biol Β· 2014
0.80
4
Association of Vitamin D Pathway Gene CYP27B1 and CYP2R1 Polymorphisms with Autoimmune Endocrine Disorders: A Meta-Analysis.
PMID: 32915988
J Clin Endocrinol Metab Β· 2020
0.70
5
Effects of CYP2R1 gene variants on vitamin D levels and status: A systematic review and meta-analysis.
PMID: 30120973
Gene Β· 2018
0.60