CDH2

Chr 18ARAD

cadherin 2

Also known as: ACOGS, ADHD8, ARVD14, CD325, CDHN, CDw325, NCAD

CDH2 encodes N-cadherin, a calcium-dependent cell adhesion protein that mediates cell-cell adhesion and is essential for proper neurite branching, synaptic organization, and dendritic spine density in neurons. Mutations cause autosomal dominant neurodevelopmental disorders with intellectual disability, developmental delay, and brain malformations. This gene is highly constrained against loss-of-function variants (pLI = 0.99, LOEUF = 0.29), indicating that haploinsufficiency is likely not tolerated in the general population.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismAR/ADLOEUF 0.293 OMIM phenotypes
Clinical SummaryCDH2
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Gene-Disease Validity (ClinGen)
arrhythmogenic right ventricular cardiomyopathy · ADLimited

Limited evidence — not for standalone diagnostic reporting

2 total gene-disease associations curated

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.99). One damaged copy is likely sufficient to cause disease.
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ClinVar Variants
52 VUS of 100 total submissions
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.29LOEUF
pLI 0.992
Z-score 5.05
OE 0.15 (0.080.29)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
2.09Z-score
OE missense 0.74 (0.680.81)
376 obs / 508.9 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.15 (0.080.29)
00.351.4
Missense OE0.74 (0.680.81)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 6 / 40.8Missense obs/exp: 376 / 508.9Syn Z: 0.17
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedCDH2-related arrhythmogenic right ventricular cardiomyopathyOTHERAD
strongCDH2-related syndromic neurodevelopmental disorder with corpus callosum, axon, cardiac, ocular, and genital defectsOTHERAD
DN
0.5082th %ile
GOF
0.6346th %ile
LOF
0.63top 25%

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

LOF1 literature citation · LOEUF 0.29

Literature Evidence

LOFCHD2 haploinsufficiency is associated with developmental delay, intellectual disability, epilepsy and neurobehavioural problemsPMID:24834135

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

100 submitted variants in ClinVar

Classification Summary

VUS52
Likely Benign23
Conflicting1
52
VUS
23
Likely Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
0
0
0
Likely Pathogenic
0
0
0
0
0
VUS
2
48
1
1
52
Likely Benign
0
1
5
17
23
Benign
0
0
0
0
0
Conflicting
1
Total24961876

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

View in ClinVar →

Protein Context — Lollipop Plot

CDH2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
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