UGT2B7

Chr 4

UDP glucuronosyltransferase family 2 member B7

Also known as: UDPGT 2B7, UDPGT 2B9, UDPGT2B7, UDPGTH2, UDPGTh-2, UGT2B9

This gene encodes UDP-glucuronosyltransferase 2B7, an enzyme that conjugates glucuronic acid to steroid hormones, bile acids, retinoic acid, and various drugs to facilitate their elimination from the body. The gene is not constrained against loss-of-function variants and mutations in UGT2B7 have not been established as a cause of Mendelian disease. A GeneReviews entry exists for this gene, suggesting potential clinical relevance that may involve pharmacogenetic considerations rather than classic genetic disease.

GeneReviewsOMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 1.44
Clinical SummaryUGT2B7
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
9 unique Pathogenic / Likely Pathogenic· 62 VUS of 83 total submissions
💊
Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
📖
GeneReview available — UGT2B7
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.44LOEUF
pLI 0.000
Z-score 0.04
OE 0.99 (0.701.44)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-1.33Z-score
OE missense 1.22 (1.121.34)
338 obs / 275.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.99 (0.701.44)
00.351.4
Missense OE1.22 (1.121.34)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 20 / 20.2Missense obs/exp: 338 / 275.9Syn Z: -0.59
DN
0.81top 10%
GOF
0.72top 25%
LOF
0.1994th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

83 submitted variants in ClinVar

Classification Summary

Pathogenic9
VUS62
Likely Benign2
9
Pathogenic
62
VUS
2
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
9
0
9
Likely Pathogenic
0
0
0
0
0
VUS
0
60
2
0
62
Likely Benign
0
1
0
1
2
Benign
0
0
0
0
0
Total06111173

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

UGT2B7 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗