DZIP3

Chr 3

DAZ interacting zinc finger protein 3

Also known as: PPP1R66, UURF2, hRUL138

DZIP3 encodes an E3 ubiquitin ligase that mediates ubiquitination and proteasomal degradation of target proteins and can specifically bind RNA. Mutations cause autosomal recessive neurodevelopmental disorder with microcephaly, epilepsy, and brain malformations. The gene is highly constrained against loss-of-function variants, indicating intolerance to complete protein loss.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.65
Clinical SummaryDZIP3
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.65LOEUF
pLI 0.000
Z-score 3.96
OE 0.48 (0.360.65)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.71Z-score
OE missense 0.92 (0.850.98)
549 obs / 598.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.48 (0.360.65)
00.351.4
Missense OE0.92 (0.850.98)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 33 / 68.4Missense obs/exp: 549 / 598.0Syn Z: 0.50

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DZIP3 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

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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