CTNND2

Chr 5

catenin delta 2

Also known as: GT24, NPRAP

The protein regulates dendritic spine and synapse formation in neurons, controls Wnt signaling through beta-catenin regulation, and functions in neuronal cell adhesion. Mutations cause autosomal recessive intellectual disability with severe speech delay, often accompanied by autism spectrum disorder and behavioral abnormalities. This gene is extremely intolerant to loss-of-function variants, indicating its critical role in neurodevelopment.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.10
Clinical SummaryCTNND2
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
113 unique Pathogenic / Likely Pathogenic· 193 VUS of 500 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.10LOEUF
pLI 1.000
Z-score 7.21
OE 0.03 (0.010.10)
Highly constrained

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

Missense Constraint
2.77Z-score
OE missense 0.70 (0.650.76)
489 obs / 694.7 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.03 (0.010.10)
00.351.4
Missense OE0.70 (0.650.76)
00.61.4
Synonymous OE1.09
01.21.6
LoF obs/exp: 2 / 64.4Missense obs/exp: 489 / 694.7Syn Z: -1.27
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongCTNND2-related neurodevelopmental disorderLOFAD
DN
0.3892th %ile
GOF
0.4677th %ile
LOF
0.80top 5%

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

LOFprediction above median · 1 literature citation · LOEUF 0.10

Literature Evidence

LOFTurner et al (2015) performed exome sequencing on 13 females with autism and identified 2 missense variants in the CTNND2 gene. To further explore variation in this gene, they sequenced CTNND2 in 362 additional autism families and identified 7 variants (additional observations of the original 2 missPMID:25807484

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

500 submitted variants in ClinVar

Classification Summary

Pathogenic96
Likely Pathogenic17
VUS193
Likely Benign89
Benign87
Conflicting8
96
Pathogenic
17
Likely Pathogenic
193
VUS
89
Likely Benign
87
Benign
8
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
3
0
93
0
96
Likely Pathogenic
8
2
7
0
17
VUS
6
152
35
0
193
Likely Benign
0
26
40
23
89
Benign
1
5
71
10
87
Conflicting
8
Total1818524633490

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

View in ClinVar →

Protein Context — Lollipop Plot

CTNND2 · 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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