ELP1

Chr 9ADARSomatic

elongator acetyltransferase complex subunit 1

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

ELP1 encodes a scaffold protein that is a component of the elongator complex required for tRNA modifications and also assembles active kinase complexes involved in proinflammatory signaling. Mutations cause familial dysautonomia, a severe neurodevelopmental disorder affecting the autonomic nervous system, and are also associated with medulloblastoma. The gene follows autosomal recessive inheritance for familial dysautonomia and shows high constraint against loss-of-function variants (LOEUF 0.735).

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismAD/AR/SomaticLOEUF 0.732 OMIM phenotypes
Clinical SummaryELP1
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 OE0.58 (0.460.73)
00.351.4
Missense OE1.01 (0.951.07)
00.61.4
Synonymous OE0.99
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.

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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