DCHS1

Chr 11ADAR

dachsous cadherin-related 1

Also known as: CDH25, CDHR6, FIB1, MMVP2, MVP2, PCDH16, VMLDS1

DCHS1 encodes a calcium-dependent cell adhesion protein that regulates neuroprogenitor cell proliferation and differentiation, and is critical for proper mitral valve morphogenesis by controlling cell migration during valve formation. Mutations cause Van Maldergem syndrome 1 and mitral valve prolapse 2, with both autosomal dominant and autosomal recessive inheritance patterns reported. This gene is highly constrained against loss-of-function variants, indicating that such variants are likely to be pathogenic.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismAD/ARLOEUF 0.242 OMIM phenotypes
Clinical SummaryDCHS1
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Gene-Disease Validity (ClinGen)
van Maldergem syndrome · ARModerate

Moderate evidence — consider for supplementary testing

2 total gene-disease associations curated

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.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.24LOEUF
pLI 1.000
Z-score 7.64
OE 0.16 (0.100.24)
Highly constrained

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

Missense Constraint
2.40Z-score
OE missense 0.85 (0.810.88)
1630 obs / 1926.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.16 (0.100.24)
00.351.4
Missense OE0.85 (0.810.88)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 15 / 95.6Missense obs/exp: 1630 / 1926.5Syn Z: 1.50

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DCHS1 · 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.

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Clinical Literature
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Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗