EIF2B4

Chr 2AR

eukaryotic translation initiation factor 2B subunit delta

Also known as: EIF-2B, EIF2B, EIF2Bdelta, VWM4

The protein functions as the delta subunit of eukaryotic initiation factor 2B (EIF2B), a GTP exchange factor essential for protein synthesis. Mutations cause leukoencephalopathy with vanishing white matter, sometimes accompanied by ovarian failure, through an autosomal recessive inheritance pattern. The high constraint scores (pLI 0.96, LOEUF 0.33) support that pathogenicity occurs predominantly through loss-of-function mechanisms affecting protein synthesis regulation.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismARLOEUF 0.331 OMIM phenotype
Clinical SummaryEIF2B4
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Gene-Disease Validity (ClinGen)
leukoencephalopathy with vanishing white matter 4 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.96). One damaged copy is likely sufficient to cause disease.
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GeneReview available — EIF2B4
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.33LOEUF
pLI 0.961
Z-score 4.18
OE 0.14 (0.070.33)
Highly constrained

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

Missense Constraint
0.80Z-score
OE missense 0.87 (0.780.96)
257 obs / 295.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.14 (0.070.33)
00.351.4
Missense OE0.87 (0.780.96)
00.61.4
Synonymous OE1.16
01.21.6
LoF obs/exp: 4 / 27.8Missense obs/exp: 257 / 295.6Syn Z: -1.35
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveEIF2B4-related leukoencephalopathy with vanishing white matterLOFAR

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

DN
0.4389th %ile
GOF
0.2895th %ile
LOF
0.66top 25%

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. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

EIF2B4 · 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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