EEF1B2

Chr 2

eukaryotic translation elongation factor 1 beta 2

Also known as: EEF1B, EEF1B1, EF1B

This gene encodes a catalytic subunit of the guanine nucleotide exchange factor that stimulates GDP-to-GTP exchange on elongation factor 1A during protein synthesis at the ribosome. Mutations cause autosomal recessive intellectual disability with seizures and language delay, typically presenting in early childhood. The gene shows moderate constraint against loss-of-function variants.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.75
Clinical SummaryEEF1B2
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.33) despite low pLI — interpret in context.
📋
ClinVar Variants
29 unique Pathogenic / Likely Pathogenic· 30 VUS of 72 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.75LOEUF
pLI 0.066
Z-score 2.17
OE 0.33 (0.160.75)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.33Z-score
OE missense 0.92 (0.791.07)
114 obs / 124.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.33 (0.160.75)
00.351.4
Missense OE0.92 (0.791.07)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 4 / 12.2Missense obs/exp: 114 / 124.3Syn Z: -0.35
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedEEF1B2-related intellectual developmental disorderLOFAR

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

DN
0.6647th %ile
GOF
0.5170th %ile
LOF
0.3067th %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

72 submitted variants in ClinVar

Classification Summary

Pathogenic28
Likely Pathogenic1
VUS30
Likely Benign3
Benign1
28
Pathogenic
1
Likely Pathogenic
30
VUS
3
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
3
0
25
0
28
Likely Pathogenic
0
0
1
0
1
VUS
0
26
4
0
30
Likely Benign
0
1
0
2
3
Benign
0
0
1
0
1
Total32731263

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

EEF1B2 · 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 →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 3 results · since 2015Search PubMed ↗