DSG2

Chr 18

desmoglein 2

Also known as: CDHF5, HDGC

This gene encodes a member of the desmoglein family and cadherin cell adhesion molecule superfamily of proteins. Desmogleins are calcium-binding transmembrane glycoprotein components of desmosomes, cell-cell junctions between epithelial, myocardial, and other cell types. The encoded preproprotein is proteolytically processed to generate the mature glycoprotein. This gene is present in a gene cluster with other desmoglein gene family members on chromosome 18. Mutations in this gene have been associated with arrhythmogenic right ventricular dysplasia, familial, 10. [provided by RefSeq, Jan 2016]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtArrhythmogenic right ventricular dysplasia, familial, 10
UniProtCardiomyopathy, dilated, 1BB

Clinical highlights

Gene-disease validity (ClinGen)
arrhythmogenic right ventricular cardiomyopathy · ADDefinitivesufficient evidence for diagnostic panels2 gene-disease associations curated in total
1
Active trials
84
Pubs (1 yr)
P/LP submissions
P/LP missense
0.79
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — DSG2
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.79LOEUF
pLI 0.000
Z-score 2.61
OE 0.55 (0.390.79)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.30Z-score
OE missense 0.97 (0.901.03)
567 obs / 587.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.55 (0.390.79)
00.351.4
Missense OE?0.97 (0.901.03)
00.61.4
Synonymous OE?0.94
01.21.6
LoF obs/exp: 22 / 39.7Missense obs/exp: 567 / 587.2Syn Z: 0.67

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DSG2 · protein map & ClinVar variants

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