UGT1A10

Chr 2

UDP glucuronosyltransferase family 1 member A10

Also known as: UGT-1J, UGT1-10, UGT1.10, UGT1J

This gene encodes UDP-glucuronosyltransferase 1A10, an enzyme that conjugates lipophilic molecules including drugs, hormones, and xenobiotics with glucuronic acid to facilitate their excretion from the body. The gene shows low constraint against loss-of-function variants (high LOEUF score of 1.742), and no Mendelian diseases have been definitively associated with UGT1A10 mutations in current databases. This enzyme is part of a gene cluster that includes UGT1A1, mutations in which cause Gilbert syndrome and Crigler-Najjar syndrome with bilirubin metabolism defects.

Summary from RefSeq, UniProt
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0
Active trials
8
Pubs (1 yr)
32
P/LP submissions
21%
P/LP missense
1.74
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryUGT1A10
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
28 unique Pathogenic / Likely Pathogenic· 295 VUS of 398 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.74LOEUF
pLI 0.000
Z-score -1.09
OE 1.26 (0.921.74)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-1.40Z-score
OE missense 1.23 (1.131.34)
366 obs / 297.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.26 (0.921.74)
00.351.4
Missense OE1.23 (1.131.34)
00.61.4
Synonymous OE1.42
01.21.6
LoF obs/exp: 25 / 19.8Missense obs/exp: 366 / 297.9Syn Z: -3.56
DN
0.74top 25%
GOF
0.72top 25%
LOF
0.2189th %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

398 submitted variants in ClinVar

Classification Summary

Pathogenic15
Likely Pathogenic13
VUS295
Likely Benign68
Benign2
Conflicting3
15
Pathogenic
13
Likely Pathogenic
295
VUS
68
Likely Benign
2
Benign
3
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
6
1
8
0
15
Likely Pathogenic
7
5
1
0
13
VUS
3
285
7
0
295
Likely Benign
0
25
10
33
68
Benign
0
1
0
1
2
Conflicting
3
Total163172634396

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

View in ClinVar →

Protein Context — Lollipop Plot

UGT1A10 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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