ECEL1

Chr 2AR

endothelin converting enzyme like 1

Also known as: DA5D, DINE, ECEX, XCE

This gene encodes a zinc-containing endopeptidase that degrades peptide hormones and inactivates neuronal peptides. Mutations cause autosomal recessive distal arthrogryposis type 5D, which involves joint contractures primarily affecting the hands and feet. The gene is not highly constrained against loss-of-function variation.

Summary from RefSeq, OMIM, UniProt
Research Assistant →

Primary Disease Associations & Inheritance

Arthrogryposis, distal, type 5DMIM #615065
AR
0
Active trials
7
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.83
LOEUF
LOF
Mechanism· G2P
Clinical SummaryECEL1
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
0.83LOEUF
pLI 0.000
Z-score 2.46
OE 0.60 (0.440.83)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.48Z-score
OE missense 0.94 (0.861.01)
409 obs / 437.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.60 (0.440.83)
00.351.4
Missense OE0.94 (0.861.01)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 27 / 44.8Missense obs/exp: 409 / 437.1Syn Z: -0.32
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveECEL1-related arthrogryposis, distalLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6649th %ile
GOF
0.74top 25%
LOF
0.2191th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ECEL1 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →