The protein is an antimicrobial host-defense peptide that binds CCR6 chemokine receptors and functions in cell chemotaxis and bacterial defense responses, with demonstrated antiplasmodial activity. Based on the available data, no specific disease associations or inheritance patterns have been established for DEFB130B mutations. The gene appears to be relatively tolerant to loss-of-function variation based on constraint metrics.

Summary from RefSeq, UniProt, Mechanism
Research Assistant →
0
Active trials
0
Pubs (1 yr)
37
P/LP submissions
P/LP missense
1.89
LOEUF
DN
Mechanism· predicted
Clinical SummaryDEFB130B
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.00) despite low pLI — interpret in context.
📋
ClinVar Variants
37 unique Pathogenic / Likely Pathogenic of 39 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.89LOEUF
pLI 0.296
Z-score 0.35
OE 0.00 (0.001.89)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.22Z-score
OE missense 0.54 (0.181.77)
1 obs / 1.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.001.89)
00.351.4
Missense OE0.54 (0.181.77)
00.61.4
Synonymous OE0.00
01.21.6
LoF obs/exp: 0 / 0.1Missense obs/exp: 1 / 1.9Syn Z: 0.67
DN
0.6841th %ile
GOF
0.4579th %ile
LOF
0.2483th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

39 submitted variants in ClinVar

Classification Summary

Pathogenic37
Benign2
37
Pathogenic
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
37
Likely Pathogenic
0
VUS
0
Likely Benign
0
Benign
2
Total39

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

DEFB130B · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found