ELAVL2

Chr 9

ELAV like RNA binding protein 2

Also known as: HEL-N1, HELN1, HUB

ELAVL2 encodes a neuron-specific RNA-binding protein that regulates gene expression by binding to AU-rich elements in the 3' untranslated regions of target mRNAs, including its own transcript and those of FOS, ID, and POU5F1. Mutations cause autosomal dominant or recessive neurodevelopmental disorders with epilepsy, intellectual disability, and movement abnormalities. The gene is highly constrained against loss-of-function variants, indicating that ELAVL2 function is critical for normal neuronal development and function.

GeneReviewsResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.19
Clinical SummaryELAVL2
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
📖
GeneReview available — ELAVL2
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.19LOEUF
pLI 0.995
Z-score 3.68
OE 0.00 (0.000.19)
Highly constrained

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

Missense Constraint
1.29Z-score
OE missense 0.74 (0.650.85)
150 obs / 201.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.19)
00.351.4
Missense OE0.74 (0.650.85)
00.61.4
Synonymous OE1.41
01.21.6
LoF obs/exp: 0 / 15.8Missense obs/exp: 150 / 201.5Syn Z: -2.73
DN
0.4091th %ile
GOF
0.3292th %ile
LOF
0.83top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.19

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ELAVL2 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →