EMILIN2

Chr 18

elastin microfibril interfacer 2

Also known as: EMILIN-2, FOAP-10

EMILIN2 encodes a protein that anchors smooth muscle cells to elastic fibers in blood vessels and contributes to elastic fiber formation and vessel assembly. Mutations cause autosomal recessive distal arthrogryposis type 10, characterized by congenital joint contractures primarily affecting the hands and feet. The gene shows no intolerance to loss-of-function variants based on population data.

Summary from RefSeq, UniProt
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0
Active trials
7
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.05
LOEUF
Mechanism
Clinical SummaryEMILIN2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.05LOEUF
pLI 0.000
Z-score 1.32
OE 0.76 (0.551.05)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.27Z-score
OE missense 0.97 (0.901.04)
518 obs / 535.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.76 (0.551.05)
00.351.4
Missense OE0.97 (0.901.04)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 26 / 34.3Missense obs/exp: 518 / 535.7Syn Z: -0.22

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

EMILIN2 · 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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