DUSP16

Chr 12

dual specificity phosphatase 16

Also known as: MKP-7, MKP7

This gene encodes a dual specificity protein phosphatase that inactivates MAP kinases by dephosphorylating both serine/threonine and tyrosine residues, specifically regulating the JNK and ERK signaling pathways. Mutations cause autosomal recessive intellectual disability with seizures and brain malformations. The gene shows significant constraint against loss-of-function variants (LOEUF 0.502), suggesting intolerance to complete protein loss.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
10
Pubs (1 yr)
44
P/LP submissions
0%
P/LP missense
0.50
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryDUSP16
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.27) despite low pLI — interpret in context.
📋
ClinVar Variants
44 unique Pathogenic / Likely Pathogenic· 76 VUS of 136 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.50LOEUF
pLI 0.089
Z-score 3.48
OE 0.27 (0.150.50)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.89Z-score
OE missense 0.87 (0.800.95)
329 obs / 377.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.27 (0.150.50)
00.351.4
Missense OE0.87 (0.800.95)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 7 / 26.2Missense obs/exp: 329 / 377.6Syn Z: -0.19
DN
0.74top 25%
GOF
0.6639th %ile
LOF
0.3940th %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

136 submitted variants in ClinVar

Classification Summary

Pathogenic42
Likely Pathogenic2
VUS76
Likely Benign4
Benign2
42
Pathogenic
2
Likely Pathogenic
76
VUS
4
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
42
0
42
Likely Pathogenic
0
0
2
0
2
VUS
0
71
5
0
76
Likely Benign
0
0
0
4
4
Benign
1
0
1
0
2
Total171504126

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

View in ClinVar →

Protein Context — Lollipop Plot

DUSP16 · 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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