WDR74

Chr 11

WD repeat domain 74

Also known as: Nsa1

The WDR74 protein regulates the MTREX-exosome complex and participates in early pre-rRNA processing steps required for 60S ribosomal subunit synthesis. Mutations cause autosomal recessive Galloway-Mowat syndrome, characterized by early-onset nephrotic syndrome, microcephaly, developmental delay, and seizures. This gene is extremely intolerant to loss-of-function mutations, reflecting its essential role in ribosome biogenesis.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.12
Clinical SummaryWDR74
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
1.12LOEUF
pLI 0.000
Z-score 1.13
OE 0.74 (0.501.12)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.92Z-score
OE missense 0.83 (0.730.93)
185 obs / 223.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.74 (0.501.12)
00.351.4
Missense OE0.83 (0.730.93)
00.61.4
Synonymous OE0.84
01.21.6
LoF obs/exp: 16 / 21.7Missense obs/exp: 185 / 223.8Syn Z: 1.19

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

WDR74 · 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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