PCDH20

Chr 13

protocadherin 20

Also known as: PCDH13

The protein is a potential calcium-dependent cell-adhesion protein containing 6 extracellular cadherin domains that is thought to establish and maintain specific cell-cell connections in the brain. Mutations cause autosomal recessive developmental and epileptic encephalopathy with microcephaly, affecting neurological development. The gene is highly constrained against loss-of-function variants (pLI 0.86, LOEUF 0.38), indicating intolerance to protein-disrupting mutations.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.38
Clinical SummaryPCDH20
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.86) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
66 unique Pathogenic / Likely Pathogenic· 140 VUS of 217 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.38LOEUF
pLI 0.862
Z-score 3.79
OE 0.17 (0.080.38)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.71Z-score
OE missense 0.91 (0.840.98)
456 obs / 500.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.17 (0.080.38)
00.351.4
Missense OE0.91 (0.840.98)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 4 / 24.1Missense obs/exp: 456 / 500.4Syn Z: -0.89

ClinVar Variant Classifications

217 submitted variants in ClinVar

Classification Summary

Pathogenic65
Likely Pathogenic1
VUS140
Likely Benign8
Conflicting1
65
Pathogenic
1
Likely Pathogenic
140
VUS
8
Likely Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
65
0
65
Likely Pathogenic
0
0
1
0
1
VUS
0
130
10
0
140
Likely Benign
0
7
1
0
8
Benign
0
0
0
0
0
Conflicting
1
Total0137770215

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

View in ClinVar →

Protein Context — Lollipop Plot

PCDH20 · 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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