NUP210

Chr 3

nucleoporin 210

Also known as: GP210, POM210

The encoded nucleoporin is essential for nuclear pore assembly, fusion, and spacing, serving as a membrane-spanning glycoprotein that maintains structural integrity of nuclear pore complexes that regulate macromolecule transport between nucleus and cytoplasm. Mutations cause neurodevelopmental disorders with intellectual disability and seizures, inherited in an autosomal recessive pattern. This gene shows strong evolutionary constraint against loss-of-function variants, indicating its critical cellular function.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.73
Clinical SummaryNUP210
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
2 unique Pathogenic / Likely Pathogenic· 143 VUS of 200 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.73LOEUF
pLI 0.000
Z-score 3.66
OE 0.58 (0.460.73)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.78Z-score
OE missense 0.93 (0.890.98)
1070 obs / 1144.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.58 (0.460.73)
00.351.4
Missense OE0.93 (0.890.98)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 50 / 86.8Missense obs/exp: 1070 / 1144.5Syn Z: -0.96

ClinVar Variant Classifications

200 submitted variants in ClinVar

Classification Summary

Pathogenic2
VUS143
Likely Benign6
2
Pathogenic
143
VUS
6
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
2
0
2
Likely Pathogenic
0
0
0
0
0
VUS
0
142
1
0
143
Likely Benign
0
5
0
1
6
Benign
0
0
0
0
0
Total014731151

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

View in ClinVar →

Protein Context — Lollipop Plot

NUP210 · 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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