WDFY4

Chr 10

WDFY family member 4

Also known as: C10orf64

The WDFY4 protein regulates cross-presentation of viral and tumor antigens in dendritic cells and plays a role in B-cell survival through autophagy regulation. Mutations cause autosomal recessive primary immunodeficiency with susceptibility to viral infections and potential tumor predisposition. The gene is highly constrained against loss-of-function variants (LOEUF 0.304), suggesting that complete protein loss is likely pathogenic.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
15
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.30
LOEUF· LoF intol.
Mechanism
Clinical SummaryWDFY4
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.23) despite low pLI — interpret in context.
📋
ClinVar Variants
156 VUS of 200 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.30LOEUF
pLI 0.340
Z-score 8.63
OE 0.23 (0.170.30)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
3.12Z-score
OE missense 0.79 (0.750.82)
1319 obs / 1679.2 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.23 (0.170.30)
00.351.4
Missense OE0.79 (0.750.82)
00.61.4
Synonymous OE0.91
01.21.6
LoF obs/exp: 33 / 145.3Missense obs/exp: 1319 / 1679.2Syn Z: 1.90

ClinVar Variant Classifications

200 submitted variants in ClinVar

Classification Summary

VUS156
Likely Benign15
156
VUS
15
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
0
0
0
Likely Pathogenic
0
0
0
0
0
VUS
0
155
1
0
156
Likely Benign
0
12
0
3
15
Benign
0
0
0
0
0
Total016713171

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

View in ClinVar →

Protein Context — Lollipop Plot

WDFY4 · 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
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