EXOC3

Chr 5

exocyst complex component 3

Also known as: SEC6, SEC6L1, Sec6p

EXOC3 encodes a component of the exocyst complex that docks exocytic vesicles with fusion sites on the plasma membrane and is essential for epithelial cell surface polarity. Mutations cause autosomal recessive developmental and epileptic encephalopathy with microcephaly. The gene is highly constrained against loss-of-function variants, reflecting its essential cellular role.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.35
Clinical SummaryEXOC3
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.92). One damaged copy is likely sufficient to cause disease.
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ClinVar Variants
48 unique Pathogenic / Likely Pathogenic· 47 VUS of 100 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.35LOEUF
pLI 0.922
Z-score 4.25
OE 0.17 (0.090.35)
Highly constrained

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

Missense Constraint
2.43Z-score
OE missense 0.68 (0.620.74)
306 obs / 451.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.17 (0.090.35)
00.351.4
Missense OE0.68 (0.620.74)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 5 / 30.2Missense obs/exp: 306 / 451.5Syn Z: -0.59

ClinVar Variant Classifications

100 submitted variants in ClinVar

Classification Summary

Pathogenic47
Likely Pathogenic1
VUS47
Likely Benign2
Benign3
47
Pathogenic
1
Likely Pathogenic
47
VUS
2
Likely Benign
3
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
47
0
47
Likely Pathogenic
0
0
1
0
1
VUS
0
39
8
0
47
Likely Benign
0
1
0
1
2
Benign
0
1
0
2
3
Total041563100

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

View in ClinVar →

Protein Context — Lollipop Plot

EXOC3 · 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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