ELAVL4

Chr 1

ELAV like RNA binding protein 4

Also known as: HUD, PNEM

This protein binds to and stabilizes mRNAs involved in neuronal development and function, particularly through binding to AU-rich elements in 3' untranslated regions of target transcripts. Mutations cause autosomal recessive late-onset distal myopathy affecting skeletal muscle, typically presenting in adulthood. The gene is highly constrained against loss-of-function variants, indicating it is essential for normal cellular function.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.38
Clinical SummaryELAVL4
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.91). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.38LOEUF
pLI 0.908
Z-score 3.31
OE 0.12 (0.050.38)
Highly constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.85Z-score
OE missense 0.46 (0.390.54)
102 obs / 221.6 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.12 (0.050.38)
00.351.4
Missense OE0.46 (0.390.54)
00.61.4
Synonymous OE0.97
01.21.6
LoF obs/exp: 2 / 16.5Missense obs/exp: 102 / 221.6Syn Z: 0.24
DN
0.4587th %ile
GOF
0.4184th %ile
LOF
0.78top 5%

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

LOFprediction above median · LOEUF 0.38

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

ELAVL4 · 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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