NKD2

Chr 5

NKD inhibitor of Wnt signaling pathway 2

Also known as: Naked2

The protein functions as a negative regulator of canonical Wnt signaling through interaction with Dishevelled proteins and is required for processing and targeting of transforming growth factor alpha to epithelial cell membranes. Mutations cause autosomal dominant developmental delay, intellectual disability, and congenital anomalies with early childhood onset. The gene shows minimal constraint against loss-of-function variants, with some tolerance for such mutations in the general population.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 1.51
Clinical SummaryNKD2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
135 unique Pathogenic / Likely Pathogenic· 121 VUS of 290 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.51LOEUF
pLI 0.000
Z-score -0.07
OE 1.02 (0.701.51)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.10Z-score
OE missense 1.02 (0.921.12)
277 obs / 272.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.02 (0.701.51)
00.351.4
Missense OE1.02 (0.921.12)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 18 / 17.7Missense obs/exp: 277 / 272.4Syn Z: 0.39
DN
0.5476th %ile
GOF
0.5268th %ile
LOF
0.66top 25%

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

LOFprediction above median

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

ClinVar Variant Classifications

290 submitted variants in ClinVar

Classification Summary

Pathogenic133
Likely Pathogenic2
VUS121
Likely Benign18
Benign5
133
Pathogenic
2
Likely Pathogenic
121
VUS
18
Likely Benign
5
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
133
0
133
Likely Pathogenic
0
0
2
0
2
VUS
0
98
23
0
121
Likely Benign
0
11
3
4
18
Benign
0
3
2
0
5
Total01121634279

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

View in ClinVar →

Protein Context — Lollipop Plot

NKD2 · 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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