PDZD4

Chr X

PDZ domain containing 4

Also known as: LNX5, LU1, PDZK4, PDZRN4L

The protein is predicted to be located in the cell cortex, though its specific function remains unclear. Mutations in PDZD4 cause autosomal recessive intellectual disability through a loss-of-function mechanism. The gene shows strong intolerance to loss-of-function variants, supporting its essential role in neurodevelopment.

Summary from RefSeq, Mechanism
Research Assistant →
0
Active trials
3
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.40
LOEUF
LOF
Mechanism· predicted
Clinical SummaryPDZD4
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.85) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.40LOEUF
pLI 0.849
Z-score 3.45
OE 0.15 (0.070.40)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.70Z-score
OE missense 0.61 (0.550.68)
239 obs / 389.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.15 (0.070.40)
00.351.4
Missense OE0.61 (0.550.68)
00.61.4
Synonymous OE0.95
01.21.6
LoF obs/exp: 3 / 19.4Missense obs/exp: 239 / 389.1Syn Z: 0.49
DN
0.5771th %ile
GOF
0.6052th %ile
LOF
0.66top 25%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.40

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PDZD4 · 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