XPO7

Chr 8

exportin 7

Also known as: EXP7, RANBP16

XPO7 encodes exportin-7, a nuclear transport receptor that mediates the export of proteins from the nucleus to the cytoplasm by forming complexes with cargo proteins and RAN-GTP. Mutations cause autosomal dominant developmental delay with variable intellectual disability and behavioral abnormalities. This gene is highly constrained against loss-of-function variants in the general population.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
9
Pubs (1 yr)
81
P/LP submissions
0%
P/LP missense
0.12
LOEUF· LoF intol.
LOF
Mechanism· predicted
Clinical SummaryXPO7
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
81 unique Pathogenic / Likely Pathogenic· 56 VUS of 188 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.12LOEUF
pLI 1.000
Z-score 7.10
OE 0.05 (0.020.12)
Highly constrained

Among the most LoF-intolerant genes (~top 3%)

Missense Constraint
4.82Z-score
OE missense 0.44 (0.400.49)
262 obs / 591.6 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.05 (0.020.12)
00.351.4
Missense OE0.44 (0.400.49)
00.61.4
Synonymous OE1.11
01.21.6
LoF obs/exp: 3 / 64.6Missense obs/exp: 262 / 591.6Syn Z: -1.27
DN
0.2798th %ile
GOF
0.4283th %ile
LOF
0.68top 10%

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

LOFprediction above median · LOEUF 0.12

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

188 submitted variants in ClinVar

Classification Summary

Pathogenic78
Likely Pathogenic3
VUS56
Likely Benign1
Benign1
78
Pathogenic
3
Likely Pathogenic
56
VUS
1
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
78
0
78
Likely Pathogenic
0
0
3
0
3
VUS
0
48
8
0
56
Likely Benign
0
0
1
0
1
Benign
0
0
0
1
1
Total048901139

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

View in ClinVar →

Protein Context — Lollipop Plot

XPO7 · 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.

Search ClinicalTrials.gov →
Clinical Literature
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗