OR1B1

Chr 9

olfactory receptor family 1 subfamily B member 1

Also known as: OR9-26, OR9-B

The protein functions as an odorant receptor involved in smell detection. Mutations in this gene cause autosomal recessive congenital anosmia, resulting in complete absence of the sense of smell from birth. The gene shows low constraint against loss-of-function variants, consistent with its role in a non-essential sensory function.

ResearchSummary from UniProt
MultiplemechanismLOEUF 1.80
Clinical SummaryOR1B1
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· 46 VUS of 79 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.80LOEUF
pLI 0.000
Z-score -0.17
OE 1.07 (0.601.80)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.04Z-score
OE missense 0.99 (0.881.12)
184 obs / 185.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.07 (0.601.80)
00.351.4
Missense OE0.99 (0.881.12)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 7 / 6.5Missense obs/exp: 184 / 185.7Syn Z: -0.13
DN
0.86top 5%
GOF
0.86top 5%
LOF
0.1499th %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

79 submitted variants in ClinVar

Classification Summary

Pathogenic27
Likely Pathogenic1
VUS46
Likely Benign3
Benign2
27
Pathogenic
1
Likely Pathogenic
46
VUS
3
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
27
0
27
Likely Pathogenic
0
0
1
0
1
VUS
0
43
3
0
46
Likely Benign
0
3
0
0
3
Benign
1
0
1
0
2
Total14632079

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

View in ClinVar →

Protein Context — Lollipop Plot

OR1B1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
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