DSC2

Chr 18ADAR

desmocollin 2

Also known as: ARVD11, CDHF2, DG2, DGII/III, DSC3

This gene encodes a member of the desmocollin protein subfamily. Desmocollins, along with desmogleins, are cadherin-like transmembrane glycoproteins that are major components of the desmosome. Desmosomes are cell-cell junctions that help resist shearing forces and are found in high concentrations in cells subject to mechanical stress. This gene is found in a cluster with other desmocollin family members on chromosome 18. Mutations in this gene are associated with arrhythmogenic right ventricular dysplasia-11, and reduced protein expression has been described in several types of cancer. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Feb 2015]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Arrhythmogenic right ventricular dysplasia 11MIM #610476
ADAR
Arrhythmogenic right ventricular dysplasia 11 with mild palmoplantar keratoderma and woolly hairMIM #610476
ADAR

Clinical highlights

Gene-disease validity (ClinGen)
familial isolated arrhythmogenic right ventricular dysplasia · ADDefinitivesufficient evidence for diagnostic panels
1
Active trials
34
Pubs (1 yr)
P/LP submissions
P/LP missense
0.69
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — DSC2
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.69LOEUF
pLI 0.000
Z-score 3.13
OE 0.47 (0.330.69)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.37Z-score
OE missense 0.95 (0.881.03)
458 obs / 481.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.47 (0.330.69)
00.351.4
Missense OE?0.95 (0.881.03)
00.61.4
Synonymous OE?1.02
01.21.6
LoF obs/exp: 19 / 40.5Missense obs/exp: 458 / 481.0Syn Z: -0.19

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DSC2 · protein map & ClinVar variants

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