MX2

Chr 21

MX dynamin like GTPase 2

Also known as: MXB

The MX2 protein is an interferon-induced dynamin-like GTPase that exhibits antiviral activity, particularly against HIV-1, by targeting viral capsids and affecting nuclear transport processes. This gene is not highly constrained against loss-of-function variants and currently has no established Mendelian disease associations in pediatric populations. While MX2 plays a role in antiviral defense and nucleocytoplasmic transport, pathogenic variants causing neurological or developmental phenotypes have not been definitively characterized.

Summary from RefSeq, UniProt
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0
Active trials
59
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.80
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryMX2
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
0.80LOEUF
pLI 0.000
Z-score 2.50
OE 0.54 (0.380.80)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.58Z-score
OE missense 0.92 (0.851.00)
393 obs / 426.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.54 (0.380.80)
00.351.4
Missense OE0.92 (0.851.00)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 19 / 34.9Missense obs/exp: 393 / 426.8Syn Z: -0.26
DN
0.84top 10%
GOF
0.6735th %ile
LOF
0.2191th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MX2 · 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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