ELP1

Chr 9ADARSomatic

elongator acetyltransferase complex subunit 1

Also known as: DYS, FD, IKAP, IKBKAP, IKI3, TOT1

The protein encoded by this gene is a scaffold protein and a regulator for three different kinases involved in proinflammatory signaling. The encoded protein can bind NF-kappa-B-inducing kinase and I-kappa-B kinases through separate domains and assemble them into an active kinase complex. Mutations in this gene have been associated with familial dysautonomia. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jan 2016]

OMIMResearchGenerating clinical summary…
LOFmechanismAD/AR/SomaticLOEUF 0.732 OMIM phenotypes
Clinical SummaryELP1
🧬
Gene-Disease Validity (ClinGen)
Riley-Day syndrome · ARModerate

Moderate evidence — consider for supplementary testing

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?
0.73LOEUF
pLI 0.000
Z-score 3.54
OE 0.58 (0.460.73)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.08Z-score
OE missense 1.01 (0.951.07)
698 obs / 692.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.58 (0.460.73)
00.351.4
Missense OE?1.01 (0.951.07)
00.61.4
Synonymous OE?0.99
01.21.6
LoF obs/exp: 47 / 81.5Missense obs/exp: 698 / 692.1Syn Z: 0.07

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ELP1 · 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 →