DEFB124

Chr 20

defensin beta 124

Also known as: DEFB-24

The DEFB124 protein is an antimicrobial beta defensin that provides antibacterial activity and enhances innate immunity, particularly in the prostate. Currently, no established pediatric neurological disorders are associated with mutations in this gene. This gene is not highly constrained against loss-of-function variants, suggesting tolerance to functional disruption.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
1
Pubs (1 yr)
16
P/LP submissions
P/LP missense
1.84
LOEUF
DN
Mechanism· predicted
Clinical SummaryDEFB124
Population Constraint (gnomAD)
Low constraint (pLI 0.11) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
16 unique Pathogenic / Likely Pathogenic· 8 VUS of 31 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.84LOEUF
pLI 0.113
Z-score 0.33
OE 0.70 (0.211.84)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.46Z-score
OE missense 0.79 (0.591.07)
30 obs / 38.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.70 (0.211.84)
00.351.4
Missense OE0.79 (0.591.07)
00.61.4
Synonymous OE0.83
01.21.6
LoF obs/exp: 1 / 1.4Missense obs/exp: 30 / 38.0Syn Z: 0.56
DN
0.76top 25%
GOF
0.5955th %ile
LOF
0.1498th %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

31 submitted variants in ClinVar

Classification Summary

Pathogenic10
Likely Pathogenic6
VUS8
Likely Benign6
10
Pathogenic
6
Likely Pathogenic
8
VUS
6
Likely Benign

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
10
Likely Pathogenic
6
VUS
8
Likely Benign
6
Benign
0
Total30

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

DEFB124 · 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 →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 2 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC