DEFB104A

Chr 8

defensin beta 104A

Also known as: BD-4, DEFB-4, DEFB104, DEFB4, hBD-4

Defensins form a family of antimicrobial and cytotoxic peptides made by neutrophils. Defensins are short, processed peptide molecules that are classified by structure into three groups: alpha-defensins, beta-defensins and theta-defensins. All beta-defensin genes are densely clustered in four to five syntenic chromosomal regions. Chromosome 8p23 contains at least two copies of the duplicated beta-defensin cluster. This duplication results in two identical copies of defensin, beta 104, DEFB104A and DEFB104B, in head-to-head orientation. This gene, DEFB104A, represents the more centromeric copy. [provided by RefSeq, Oct 2014]

0
Active trials
89
Pathogenic / LP
211
ClinVar variants
0
Pubs (1 yr)
0.2
Missense Z
1.89
LOEUF
Clinical SummaryDEFB104A
Population Constraint (gnomAD)
Low constraint (pLI 0.09) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
89 Pathogenic / Likely Pathogenic· 15 VUS of 211 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.89LOEUF
pLI 0.094
Z-score 0.04
OE 0.95 (0.261.89)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.20Z-score
OE missense 0.90 (0.661.23)
28 obs / 31.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.95 (0.261.89)
00.351.4
Missense OE0.90 (0.661.23)
00.61.4
Synonymous OE0.72
01.21.6
LoF obs/exp: 1 / 1.0Missense obs/exp: 28 / 31.2Syn Z: 0.74

ClinVar Variant Classifications

211 submitted variants in ClinVar

Classification Summary

Pathogenic86
Likely Pathogenic3
VUS15
Likely Benign1
Benign105
Conflicting1
86
Pathogenic
3
Likely Pathogenic
15
VUS
1
Likely Benign
105
Benign
1
Conflicting

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
86
Likely Pathogenic
3
VUS
15
Likely Benign
1
Benign
105
Conflicting
1
Total211

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

DEFB104A · 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
Landmark / reviewRecent case evidence
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found