NXT1

Chr 20

nuclear transport factor 2 like export factor 1

Also known as: MTR2, P15

The protein functions as a nuclear export factor that stimulates export of proteins containing nuclear export signals and facilitates export of various RNAs including U1 snRNA, tRNA, and mRNA through both CRM1-dependent and independent pathways. Mutations in NXT1 cause autosomal recessive intellectual disability with microcephaly and seizures, typically presenting in infancy or early childhood. The gene shows moderate constraint against loss-of-function variants, suggesting haploinsufficiency may be tolerated but complete loss of function is likely pathogenic.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
5
Pubs (1 yr)
21
P/LP submissions
0%
P/LP missense
0.89
LOEUF
Mechanism
Clinical SummaryNXT1
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.65) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
21 unique Pathogenic / Likely Pathogenic· 12 VUS of 35 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.89LOEUF
pLI 0.646
Z-score 1.69
OE 0.00 (0.000.89)
Moderately constrained

Typical tolerance to LoF variation

Missense Constraint
1.95Z-score
OE missense 0.40 (0.310.54)
34 obs / 84.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.89)
00.351.4
Missense OE0.40 (0.310.54)
00.61.4
Synonymous OE0.79
01.21.6
LoF obs/exp: 0 / 3.3Missense obs/exp: 34 / 84.4Syn Z: 0.92

ClinVar Variant Classifications

35 submitted variants in ClinVar

Classification Summary

Pathogenic18
Likely Pathogenic3
VUS12
18
Pathogenic
3
Likely Pathogenic
12
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
18
0
18
Likely Pathogenic
0
0
3
0
3
VUS
0
11
1
0
12
Likely Benign
0
0
0
0
0
Benign
0
0
0
0
0
Total01122033

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

View in ClinVar →

Protein Context — Lollipop Plot

NXT1 · 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.

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Clinical Literature
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