SELENBP1

Chr 1AR

selenium binding protein 1

Also known as: EHMTO, HEL-S-134P, LPSB, MTO, SBP56, SP56, hSBP

This protein catalyzes the oxidation of methanethiol, an organosulfur compound produced by gut bacteria, and functions as a selenium-binding protein involved in sensing reactive xenobiotics. Mutations cause extraoral halitosis due to methanethiol oxidase deficiency, inherited in an autosomal recessive pattern. The gene shows low constraint to loss-of-function variation, consistent with a recessive disorder affecting a specific metabolic pathway.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 1.291 OMIM phenotype
Clinical SummarySELENBP1
🧬
Gene-Disease Validity (ClinGen)
extraoral halitosis due to methanethiol oxidase deficiency · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
17 unique Pathogenic / Likely Pathogenic· 79 VUS of 142 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.29LOEUF
pLI 0.000
Z-score 0.35
OE 0.93 (0.671.29)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.45Z-score
OE missense 0.92 (0.831.02)
262 obs / 283.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.93 (0.671.29)
00.351.4
Missense OE0.92 (0.831.02)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 25 / 27.0Missense obs/exp: 262 / 283.3Syn Z: -0.22

ClinVar Variant Classifications

142 submitted variants in ClinVar

Classification Summary

Pathogenic13
Likely Pathogenic4
VUS79
Likely Benign9
Benign9
13
Pathogenic
4
Likely Pathogenic
79
VUS
9
Likely Benign
9
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
1
0
12
0
13
Likely Pathogenic
1
2
1
0
4
VUS
0
75
4
0
79
Likely Benign
0
1
4
4
9
Benign
0
3
3
3
9
Total281247114

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

View in ClinVar →

Protein Context — Lollipop Plot

SELENBP1 · 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.

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Clinical Literature
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